This paper describes an unusual case of ileal atresia associated with an omphalomesenteric duct (OMD) remnant. A 1-day-old male developed bilious vomiting and abdominal distension. At laparotomy, the distal portion of the ileum was connected to the umbilicus with a fibrous band. Around this omphalomesenteric band, the ileum was strangulated and necrotic; the end of the ileum was atretic. Apart from these structures, the cecum terminated without mesentery. This experience shows evidence of an OMD remnant as a possible cause of jejunoileal atresia.