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Polycystic kidney is a rare congenital malformation which consists of numerous cysts of varialbe size. The origin of the polycystic kidney is obscure but cysts in kidneys may result either from a primary disturbance in function of ampullae of colleting tubules or from a secondary enlargement of portions of collecting tubules or nephrons. Recently wer experienced a typical case of potte`s type IIa polycystic kidney associated with oligohydramnios, cebocephalus, VSD, and trisomy-D. We report this case with a brief review of its literatures available