Intestinal atresia is a congenital anomaly commonly happening in the small bowel and rarely in the colon. Colonic atresia can manifest as intestinal obstruction with abdominal distention and bilious vomiting.

Methods: A 3-day-old male new-born who was referred from a rural hospital, full term, product of normal vaginal delivery, with a weight of 2400 g. The patient had a complaint of bilious vomiting, inability to pass meconium, and abdominal distension for three days. On clinical examination the patient had visible bowel loops and yellowish aspiration from the orogastric tube. An erect abdominal radiograph showed distended bowel loops and sharp air-fluid levels. Administration of contrast enema revealed resistance to pass the rectal tube and stopping of contrast pas the rectosigmoid region with contrast spillage from the anus. Sigmoid atresia is a rare congenital anomaly that occurs in approximately 1 in 20,000 live births. The most common presentation is bilious vomiting and abdominal distension in the first 24 h of life. Diagnosis is confirmed with abdominal X-ray and contrast enema. Treatment is surgical, with primary repair being the most common approach. He underwent two stages of repair with an end colostomy and mucous fistula, then closure of the colostomy after four months of the first stage.

Conclusions: This presentation requires clinical suspicion with prompt care, as this condition can mimic Hirschsprung's disease. In our case, the patient has a classical presentation of sigmoid atresia that had an early diagnosis, which resulted in a better outcome.