Abstract

Persistent Müllerian duct syndrome is a rare disease, genotypically affects only males (46, XY); Difficult to diagnose, in adulthood patients are normally virilized, the discovery is often fortuitous during surgery to treat intra-abdominal testicular ectopia or during the management of infertility as a couple. Infertility is a common manifestation of the disease. In general, the serum testosterone concentration is normal. In children, surgical treatment is aimed at preserving testicular function, in adulthood the risk of cancerization on the intra abdominal testes is real, the excision of the testes and the müllerien remains is frequently proposed.

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