Persistent Mullerian Duct Syndrome with torsion of an intra-abdominal seminoma

Persistent Mullerian Duct Syndrome (PMDS) is a particular form of male pseudohermaphroditism. Due to the absence or inactivity of Mullerian Inhibiting Substance (MIS), no regression is observed of the Mullerian 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.