Agenesis of Urinary bladder is an extremely rare congenital genitourinary anomaly and is associated with other severe malformations that are incompatible with life. About 60 cases and 22 live births only have been reported in English literature. We report an eight year old girl with triad of complete agenesis of bladder and urethra, solitary functioning left kidney and an ectopic ureter opening into the vagina. The management of the child along with embryo-pathogenesis and literature review is discussed.
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