Renal angiomyolipoma (AML) is a benign mesenchymal neoplasm with a classic triphasic histology, adipose tissue, epithelioid smooth-muscle—like cells, and abnormally thick-walled blood vessels.1–3 Although most AMLs occur in the kidneys, they also occur in other sites, such as the liver, lungs, uterus, vagina, ovaries, colon, lymph nodes, skin, bone, nasal and/or oral cavities, retroperitoneum, and adrenal glands.2,3 Epithelioid AML (eAML) is a rare, recently recognized subtype with a natural history of malignancy. There is little knowledge about the origin, treatment options, clinical course, and imaging evaluation for these aggressive tumors.2,4–6 Inhibitors of the mammalian target of rapamycin (mTOR) protein were recently approved by the US Food and Drug Administration for various tuberous sclerosis—related lesions, including AML.7 Genetic analyses also reported mTOR activity in non—tuberous sclerosis-related AMLs. These studies provide functional evidence that mTOR activation is common to sporadic, non—tuberous sclerosis-related AMLs,8 which suggests the possibility that mTOR inhibitors such as everolimus and temsirolimus may be therapeutic for this class of disease. This article focuses on 3 aims: (1) to highlight the fluorine-18 fluorodeoxyglucose (FDG) avidity of these tumors on positron emission tomography (PET) computed tomography (CT), which, to our knowledge, has not been previously reported, (2) to demonstrate the response of eAML to mTOR inhibitors, as suggested by other case reports, and (3) to show the concomitant occurrence of peripheral T-cell lymphoma (PTCL) in association with recurrent eAML.