A case of a female neonate with congenital ileal atresia with a giant abdominal mass accompanied by calcification was reported. The giant abdominal mass was a unilocular cyst fulled with thick fluid and attached to the blind end of ileal atresia. The cyst was removed and a ileostomy was performed. The ileostomy was closed four months later and the patient was cured. In this case, there was no perforation of the alimentary tract, and the wall of the cyst was devoid of mucosal epithelium, and consisted of intestinal wall with marked granulation. There was also submucosal diffuse calcification and a strong response of atypical giant cells.
