Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare and specific entity referred to as Herlyn-Werner-Wunderlich syndrome. It usually presents after menarche with remittent pelvic pain and a palpable pelvic mass due to hematocolpos. It rarely presents with primary infertility in early adulthood. In this report, a case of Herlyn-Werner-Wunderlich syndrome with primary infertility in a young woman is described with MR imaging findings.