Persistent Mullerian Duct Syndrome in an Adult Infertile Male: A Case Report

Persistent Müllerian duct syndrome(PMDS) is a rare autosomal recessive disorder of sex development characterized by the presence of Müllerian duct derived structures in a normally virilized, genotypical (46, XY) and phenotypical male. Here we describe a case of a couple who presented as primary subfertility with azoospermia and diagnosed as PMDS (Bilateral testis in right inguinal canal with associated ring inguinal hernia and persistent of mullerian duct derivatives in the form of rudimentary uterus and fallopian tubes) and planned for Intrauterine insemination with donor semen. PMDS is rare disease and in developing countries like Nepal, because of unaware of the disease condition and lack of proper health care facilities, such cases are diagnosed later in adult males. Therefore, this case highlights the importance of awareness and knowledge for early detection and treatment of such conditions to conserve fertility and prevent malignancy of testis and other remnant mullerian structures.