Abstract Background Uterine didelphys with obstructed hemivagina is an extremely rare condition. This diagnosis should be considered whenever an adolescent is presented with severe dysmenorrhea and progressive pelvic mass. Case A 15-year-old girl presented with uterus didelphys with obstructed left hemivagina and ipsilateral renal agenesis. Excision of the septum was done using monopolar diathermy in the cutting mode under laparoscopic and hysteroscopic guidance, taking into consideration the integrity of the hymen. Complete recovery was achieved within one week. Conclusion The prompt and accurate diagnosis of this condition is essential to prevent complications from acute illness and to preserve future fertility. Ultrasonography, CT scan and MRI are the initial steps for its diagnosis; however, laparoscopy is a crucial tool for confirmation of the diagnosis. In order to avoid the associated morbidity of delayed management, surgical intervention should be considered as the first line therapy, taking into consideration the social and traditional backgrounds of the parents and patients.