Cloacal dysgenesis is a rare anomaly. Fifty cases have been reported in the literature. This condition differs from the usual imperforate anus. The diagnosis, evaluation, and therapeutic management require an understanding of the embryogenesis of the cloaca. The presence at birth of a single interlabial orifice or an abnormality sited anus is a surgical emergency. Early salvage of the kidneys and relieving intestinal obstruction are emphasized. There is a high incidence of multiple system congenital abnormalities.