This patient presented with a history of secondary amenorrhea and a central pelvic mass. She had previously failed a progesterone challenge test. Examination confirmed the mass and showed a blind vaginal pouch. During exploratory laparotomy, the definitive diagnosis of müllerian agenesis was made, and the pelvic mass was determined to be leiomyoma within the uterine anlagen. A description of the surgical procedure and a discussion on the diagnosis and management of this patient is found in this report.