Abstract. Angiomyolipoma is a benign mesenchymal tumor, most commonly arising from the kidney. With the exception of the liver, extrarenal angiomyolipoma is an infrequent entity that may be misdiagnosed as other tumors. Angiomyolipoma occurring in the skeleton is an extremely rare occurrence and often difficult to differentiate from other bone diseases. We herein present a case of angiomyolipoma in the rib of a 44-year-old male patient, a highly unusual location for this type of tumor. Angiomyolipoma of the skeleton usually exhibits an infiltrative tendency. Although this clinical entity is rare, angiomyolipoma should be considered in the differential diagnosis of skeletal lesions, particularly in patients presenting with imaging findings of fat within the lesions.
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