Persistent Müllerian Duct Syndrome with Torsion of an Intra-Abdominal Seminoma

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.Persistent Müllerian Duct Syndrome (PMDS) is a particular form of male pseudohermaphroditism. Due to the absence or inactivity of Müllerian Inhibiting Substance (MIS), no regression is observed of the Müllerian ducts in a genotypical and phenotypical male individual. This leads to the development of fallopian tubes, uterus and proximal vagina. The testes often lie intra-abdominally and are exposed to malignant degeneration. A case is described in which the diagnosis of PMDS was made by laparotomy for an acute abdomen, caused by torsion of a seminoma.