Late presentation of Mullerian agenesis as primary subfertility

Mullerian agenesis with primary amenorrhea is a rare condition and uncommonly presented as primary subfertility. It is a complex defect in the female genital tract’s embryological development. We present a case report of a 34-year-old woman presented initially for primary subfertility management, and a systematic clinical approach such as history, examination and investigations (diagnostic laparoscopy) diagnosed Mullerian agenesis with normal development of other systems. After diagnosis, psychological counselling, vaginal dilators to improve her vaginal length and adoption were done with the multidisciplinary team approach. Community-based case study assessment and early detection of primary amenorrhea with available facilities in a low resource setting are essential elements for an early diagnosis and subsequent optimal outcomes.