A 50-year-old man presented with nausea and abdominal bloating sensation of a few months' duration and was found to have a large adrenal mass that was resected after computed tomography scan evaluation. The histopathologic examination showed epithelioid and spindle cells having clear to granular eosinophilic cytoplasm, and small, central, round to oval nuclei with small nucleoli. The tumor cells demonstrated reactivity for smooth muscle actin, HMB-45, Melan-A, and vimentin, with focal positivity for desmin and S100 protein and negative staining for inhibin, synaptophysin, and chromogranin. A diagnosis of perivascular epithelioid cell tumor was made. Perivascular epithelioid cell tumors can arise in a variety of locations, including omentum/mesentery, female genital tract, pelvic soft tissues, skin, and falciform ligament, but an adrenal primary tumor has not been previously reported, to the best of our knowledge. The differential diagnosis is broad and site-dependant and in our case includes several neoplasms with clear cells that can involve the adrenal gland, including a primary adrenocortical neoplasm. A review of the related literature and discussion of the differential diagnoses are presented.