Continent cutaneous diversion and external genitalia reconstruction in a child with severe variety urogenital sinus and ambiguous genitalia

The diagnosis and management of a child with ambiguous genitalia and severe variety of urogenital sinus with a high vesico-vaginal confluence is challenging. This 4-year-old female child had solitary right kidney with ectopic ureter opening in high variety of urogenital sinus with hypo-plastic urinary bladder and incontinence. We describe genitourinary reconstruction with complete functional rehabilitation in this child. This complex problem was managed with continent urinary diversion with Penn pouch and refashioning of external genitalia, rendering continence and near normal female external genitalia. The child and parents are happy with continence and aesthetically normal external genitalia.