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Abstract:
A ten year old boy was operated for left inguinal hernia at age four years. Male gender was confirmed by sex chromatin. Y corpuscle and male genotype in chromosomal studies, and testicular function was tested by normal testosterone serum levels before and after parenteral human chorionic gonadotropin stimulus. On a second operation, two structurally normal testes and was deferens coexisted with Müllerian structures (rudimentary uterus and two Fallopian tubes of normal histological features) were found. A diagnosis of uterus-inguinalis hernia was made. Deficiencies at Müllerian inhibiting factor's secretion, activity or receptors have been postulated to explain this anomaly.