Persistent Mullerian duct syndrome (PMDS) is a rare disorder of male sexual development. It is characterized by the presence of a uterus, fallopian tubes, and upper vagina in an otherwise phenotypically and genotypically normal male. This malformation is usually an incidental finding during the operative treatment of other more common abnormalities such as inguinal hernia or undescended testes. Not uncommonly, it is seen in association with transverse testicular ectopia. This report describes a case of PMDS in association with transverse testicular ectopia diagnosed at the time of laparoscopy for undescended testes. Physicians caring for these patients should be aware of this and surgeons should be familiar with the different surgical options. PMDS should be considered in all cases of bilateral undescended testes. Aspects of diagnosis and management are also discussed.