Transverse vaginal septum is a rare occurrence. When it co-exists with cervical dysgenesis, it is even rarer. Primary amenorrhea with cyclic pain is one manifestation in post-pubertal women. A case of transverse vaginal septum with vaginal atresia and cervical dysgenesis is presented. Presentation as a huge abdominal mass and severe anaemia posed diagnostic challenges. A two-staged management is described as well as variation in management of the septum. The involvement of the patient in her follow up is also stressed.
Keywords: Primary amenorrhea; abdominal mass; cervical dysgenesis; vaginal septum.