Abstract
Embryonal Rhabdomyosarcoma ‘Botryoid Type’ is a rare soft tissue tumor that arises within the wall of the bladder or vagina and seen almost exclusively in infants. Treatment is combination of surgery with adjuvants chemotherapy and radiotherapy. We report a case of an 18-month-old Saudi girl presented with history of abdo-pelvic mass, bleeding per vagina and a mass protruding through the introitus. Computed tomography (CT) done reveling large heterogeneous pelvic mass arising from the uterus, biopsy of mass confirmed the diagnosis (sarcomabotryoidal). The patient received multiple cycles of combination chemotherapy as a neoadjuvant treatment then underwent total abdominal hysterectomy with upper vaginectomy. Postoperatively, the patient received more cycles of chemotherapy. CT scan for post-treatment evaluation was done and showed no evidence of local recurrence or distant metastasis. Currently, she is disease-free on remission with no complains.