Intestinal obstruction is common in neonates; however, Intestinal obstruction due to sigmoid atresia is very rare. We present a case of sigmoid atresia, suspected from clinical examination and radiological investigations on day two of life, and subsequently confirmed at surgery and histopathology. Sigmoid atresia Type 3(a) was noticed peroperatively. Resection and primary anastomosis was done with proximal covering stoma. The patient was discharged on 7th post operative day without any complications. Key words: Neonate, Intestinal atresia, Sigmoid colon. This article may be cited as: Imran N, Rehman IU, Waheed T. Sigmoid atresia: A rare cause of neonatal intestinal obstruction. KUST Med J 2009; 1(2): 71-72.