Congenital agenesis of the scrotum and labia majora is very exceptional. To date, only 6 cases of scrotal agenesis have been reported. To our knowledge, the anomalies of the labioscrotal folds in siblings have not yet been reported. We report the complete agenesis of the scrotum and labia majora within the 3 members of the same family. Additionally, successful reconstruction of the neoscrotum was performed for the first time for congenital scrotal agenesis. UROLOGY 81: 421-423, 2013. Crown Copyright (C) 2013 Published by Elsevier Inc.