Abstract Hirschsprung disease (HD) is usually presented early in the neonatal period with delayed or no passage of meconium up to 48 hours, with abdominal distension and bilious vomiting. A 3-month-old baby boy with a history of neonatal asphyxia presented a 2-week duration of umbilical redness and a foul-smelling greenish discharge. He was treated for omphalitis and was given oral antibiotics, but later developed feeding intolerance and abdominal distension with no bowel opening. The abdomen was grossly distended, tense with erythematous umbilicus, and a greenish discharge from the umbilical punctum. A plain abdominal radiography showed dilated bowels. With the impression of complicated patent vitello-intestinal duct, the baby was subjected for an emergency laparotomy. Intraoperatively, there was a grossly dilated and inflamed sigmoid colon with a 5 mm perforation on the sigmoid colon. It formed a direct communication to the umbilical defect, forming a colo-umbilical fistula. The perforation site was resected and brought out as a sigmoid colostomy. The resected perforation site showed an acute inflammation with the presence of ganglionic cells, histologically. Since HD is highly suspicious, a suction rectal biopsy was performed postoperatively and revealed typical features of HD. Subsequently, the patient underwent the Duhamel procedure; the stoma was brought down and anastomosed with the anal canal. Despite the typical presentation of HD, it can also present enterocolitis, and to some extent, a bowel perforation. In this case, we were fortunate that the bowel perforation formed a direct fistulation to the umbilicus, instead of free perforation inside the peritoneal cavity, causing peritonitis or a less favourable outcome.