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ABSTRACT Background: Alveolar rhabdomyosarcoma (ARMS) of the corpus uteri is a very uncommon malignant tumour that has rapid progression and a poor prognosis particularly in young adults. Guidelines and data regarding treatment are limited and usually applied by extrapolating from protocols for treating sarcomas from non-uterine sites and from guidelines for paediatric sarcomas. Case: A 21-year-old nulliparous female was evaluated for an abdominopelvic mass. Examination findings were a large abdominopelvic mass. An exploratory laparotomy was done. Massive intraoperative bleeding from a friable uterine mass with adhesions necessitated a subtotal hysterectomy. Histology confirmed a uterine alveolar rhabdomyosarcoma. The patient was planned for adjuvant chemotherapy postoperatively with the VAC regimen but follow up was a challenge due to financial challenges. Conclusion: This case report describes a rare cause of an abdominopelvic mass in a young adult of reproductive age due to an alveolar rhabdomyosarcoma and discusses therapeutic options available.