Three female patients with hypoplasia of the urinary bladder and associated congenital anomalies are described. All were first seen in early infancy with significant obstructive uropathy and were managed by early loop cutaneous ureterostomy. All have survived to adulthood and 2 patients have received a kidney transplant into an ileal conduit; 1 patient continues to do well with cutaneous ureterostomy only. Pathogenesis, management, and long-term outcome are discussed.
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