1 Departments of Pediatrics and Pathology, The George Washington University, School of Medicine, 901 23rd Street, N.W., Washington, D.C. 20037
Three male siblings with polycystic kidney disease, internal hydrocephalus, and polydactylism are presented. These identical anomalies in three siblings constitute a new syndrome. It is recommended that future studies on polycystic kidney disease with central nervous system anomalies occurring in siblings should be reported in detail if the pathogenesis of the disease is to be understood.