BackgroundExtrarenal retroperitoneal angiomyolipomas are benign tumours of mesenchymal tissue origin. The diagnosis of these tumours is usually based on the perioperative findings and confirmed by the resultant specimen's histology. The main differential diagnosis is liposarcoma, and accurate preoperative imaging combined with fine-needle aspiration cytology may permit a non-operative approach to these tumours.Case presentationWe describe a single case of extrarenal retroperitoneal pelvic angiomyolipoma and provide the first case of magnetic resonance imaging of these extremely rare tumours.ConclusionThis article discusses the presentation, management, and outcome associated with these tumours.