| Summary: | Ambiguous genitalia represents a rare but important group of disorders. This genital anomaly commonly presents with an abnormal external genitalia with or without associated metabolic complications. There is a need for early diagnosis so that appropriate sex of rearing can be assigned early in life.
Our 22 year old patient who was intially reared as a female is presented to underscore the diaster of inappropriate sex choice early in life. On presentation, he had an amenorrheic female phenotype with male secondary sex characteristics. There were bilateral undescended testes at the external inguinal rings, fused phallus and labioscrotal folds, with the urethral orifice between them. At surgery, there was defficient corpus spongiosum, with lots of cavernisal tissues folded below the pubic symphysis. Bilateral orchidopexy and one-stage genitoplasty were done and the patient now has a male-looking external genitalia.
The awareness in our environment, psychological and social embarrassment, limitations to management and the role of surgery in sex assignment are highlighted.
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