The case of a 16-year-old patient with incomplete testicular feminization syndrome is reported. The patient had phenotypically female genitalia except for clitoromegaly. Hormonal examinations revealed low plasma testosterone levels but a normal concentration of 5α-dihydrotestosterone, and radiological and surgical examinations revealed no development of Müllerian structures including the uterus and the adnexa. This happened in a family with a frequent occurrence of both hypospadias and cryptorchism.
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