ABSTRAK Penyakit Hirschsprung adalah penyebab obstruksi usus bagian bawah yang paling umum pada neonatus (0-28 hari), dengan insidens 1:5000 kelahiran hidup. Penyakit Hirschsprung lebih sering ditemukan pada laki-laki dibandingkan perempuan dengan perbandingan 4:1. Angka kematian untuk penyakit Hirschsprung berkisar antara 1-10%. Untuk mengetahui karakteristik bayi yang menderita penyakit Hirschsprung, dilakukan penelitian di RSUP H. Adam Malik Medan dengan desain case series. Populasi dan sampel penelitian berjumlah 110 orang pada tahun 2010-2012 yang tercatat di rekam medis rumah sakit. Data univariat dianalisis secara deskriptif sedangkan data bivariat dianalisis dengan menggunakan uji Chi-square, MannWhitney, Kruskal Wallis test. Proporsi berdasarkan sosiodemografi tertinggi pada kelompok umur 0-28 hari (60,0%), laki-laki (72,7%), luar Kota Medan (85,5%), rujukan Rumah Sakit Umum Kabupaten/Kota (51,8%). Proporsi berdasarkan status rawatan tertinggi adalah keluhan utama perut membesar (56,4%), gambaran klinis distensi abdomen, sulit/tidak BAB (Buang Air Besar), keterlambatan mekonium (44,5%), pemeriksaan penunjang enema barium (44,6%), penatalaksanaan medis non-bedah (59,1%), tidak ada komplikasi (80,0%), sumber biaya Jamkesmas (42,7%), lama rawatan rata-rata 14 hari dan keadaan sewaktu pulang pulang berobat jalan (43,6%) Ada perbedaan proporsi yang bermakna antara umur berdasarkan penatalaksanaan medis (p=0,000), lama rawatan berdasarkan penatalaksanaan medis (p=0,000), penatalaksanaan medis berdasarkan keadaan sewaktu pulang (p=0,000), serta lama rawatan berdasarkan keadaan sewaktu pulang (p=0,000). Tidak ada perbedaan proporsi yang bermakna antara umur berdasarkan jenis kelamin, komplikasi berdasarkan penatalaksanaan medis, serta lama rawatan rata-rata berdasarkan komplikasi. Kelangsungan hidup bayi yang menderita penyakit Hirschsprung sangat bergantung pada diagnosis awal dan pendekatan operasi sehingga perlu diberi pemahaman kepada orang tua mengenai penatalaksanaan medis terbaik untuk penyakit ini. Kata Kunci : Penyakit Hirschsprung, Karakteristik Bayi, RSUP H. Adam Malik Medan Universitas Sumatera Utara ABSTRACT Hirschsprung disease is a cause of lower intestinal obstruction most commonly in neonates (0-28 days), the incidence 1:5000 live births. Hirschsprung disease is more common in males than females with a ratio of 4:1. The mortality rate for Hirschsprung disease ranged from 1-10%. To determine the characteristics of baby suffering from Hirschsprung disease, conducted a research at RSUP H. Adam Malik Medan with case series design. Population and sample was 110 patients in 2010-2012 and recorded in hospital medical records. Univariate data were analyzed descriptively while bivariate data were analyzed using Chi-square, Mann-Whitney, Kruskal Wallis test. Based on sosiodemographic, the highest population is in the age group of 0-28 days (60,0%), male (72,7%%), and came from out of Medan area (85,5%), and referall from Public Hospital District/City (51,8%). Based on the treatment, abdominal distension (56,4%), clinical presentation of abdominal distension, difficult to defecate, delayed passage of meconium (44,5%), test of barium enema (44,6%), medical management of no surgical (59,1%), no complications (80,0%), Jamkesmas (42,7%), the average length of stay 14 days, outpatient control (43,6%). There is a significant differentiation of proportion between age based on medical management (p=0,000), average length of stay based on medical management (p=0,000), medical management based on condition when go home (p=0,000), and average length of stay based on condition when go home (p=0,000). There is no significant differentiation of proportion between age based on sex, complications based on medical management, and average length of stay based on complications. Prognosis of Hirschsprung disease depends on early diagnosed ang surgery approach so that the parents should be given the understanding of the best medical treatment for this disease. Key words : Hirschsprung disease, characterictics of baby, RSUP H. Adam Malik Medan Universitas Sumatera Utara