Anastomosing hemangioma of the genitourinary tract is a recently described vascular lesion of the kidneys and testes. Although these lesions can occur in the renal hilum with infiltration of the hilar adipose tissue, the presence of adipocytes in this type of vascular tumor has not been reported. We described a case of an anastomosing hemangioma associated with fatty changes of the stromal component in a 59-year-old female patient. This unusual morphologic finding not only represents a novel curiosity in this rare type of vascular lesion, but also could potentially pose significant diagnostic challenges as it is commonly mistaken as an angiomyolipoma.