Renal dysplasia with sacral-perineal location.

We report a case of renal dysplasia associated with a large non-renal lumbosacral and perineal mass. A huge sacral-perineal mass and malformation of the scrotum and penis were noted at birth. The mass was thought to be a lumbosacral teratoma. The patient also suffered from respiratory distress and he died 5 days after birth. At autopsy 2 small dysplastic kidneys were found. The kidneys and the sacral mass showed the same histological features of typical renal dysplasia.