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Three cases of segmental dilatation of ileum surgically are presented. Authors have reviewed medical literature since the first case reported in 1959, and have been able to collect 33 cases. Although it is considered an identical entity involving either colon or small bowel, clinical picture and age of presentation are different. Cases involving the colon have a clinical picture very similar to that of Hirschsprung's disease, usually appearing in the lactancy or later on. Cases involving ileum or jejunum usually are manifested as a neonatal bowel obstruction. Pathological examination shows a bowel wall with all his layers as well as a normal ganglion cells. Surgical resection of dilated intestinal segment is curative.
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