Adult polycystic renal disease presenting in infancy: a report emphasizing the bilateral involvement.

We describe a girl with adult polycystic renal disease that presented as a unilateral abdominal mass on an excretory urogram. The child had a 4-generation family history of this disorder. Clinical course had been unremarkable until she was 12 years old and died of a ruptured intracranial aneurysm. Despite the clinical impression of unilateral renal involvement, postmortem examination showed that the clinically normal kidney had developed early cyst formation in several areas, thus, underscoring the bilateral nature of adult polycystic disease.