Introduction: Angiomyolipoma (AML) is the most prevalent renal mesenchymal neoplasm that almost always involves kidneys.Case presentation: We present a rare condition of retroperitoneal extra-renal angiomyolipoma which relapsed after 4 years that caused acute abdominal pain.Discussion: The liver is the most common site of extrarenal angiomyolipoma. Abdominal pain is the most common complaint of extra-renal angiomyolipoma. Computed tomography angiography is the best way for angiomyolipoma diagnosis and surgery and post-surgery chemotherapy is the way for its treatment.Conclusion: extrarenal angiomyolipoma is an extremely rare condition that patients should be followed up for over 5 years by appropriate radiologic imaging.