Introduction. Angiomyolipoma is a benign tumor composed of mature fat cells, thick-walled blood vessels, and muscle cells. Angiomyolipoma is the most common renal mesenchymal neoplasm described by perivascular epithelioid cells that coexpress smooth muscle and melanogenesis markers. Retroperitoneal involvement is rare. It is quite difficult to diagnose such a tumor before surgery because such a location is more common for testicular tumors, lymphomas, leiomyosarcomas, and schwannomas. Case report. The patient, 59 years old, went to the Oncology Institute with complaints of epigastric pain in the belt, general weakness, and weight loss of about 4 kg, she is considered ill for about a month when the itching and dark urine appeared. According to the MRI data, a volume formation is visualized on the lower contour of the pancreatic head with the effect of compressing the medial duodenal wall. Excision of the 3x2x1.5 cm tumor is performed, and the histopathological examination with the conclusion of retroperitoneal angiomyolipoma, SMA (+), caldesmon (+patchy), ERG(-), CD68(-), S100(-), ER (-), Ki-67-3%. Conclusion. Angiomyolipoma is a rare benign mesenchymal tumour, that preferentially reaches the kidney, however the extra-renal location is possible, but extremely rare.