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Reports on the occurrence of persistent mullerian duct syndrome along with transverse testicular ectopia are extremely rare globally. In this condition, the fallopian tubes, uterus, cervix and upper two-thirds of vagina occurs alongside transverse testicular ectopia. In most cases, the condition is discovered as an incidental finding during surgical procedures. In this report, we present a case of a 29-year-old Somali male that had scrotal swelling, pain around the inguinal area and perceived infertility for over seven years whom we incidentally found to have a persistent mullerian duct syndrome with transverse testicular ectopia and managed accordingly.