A 52-year-old female was referred to our institute for an incidental renal mass. A homogenously enhancing mass was detected on multidetector CT (MDCT) imaging. Histologically, the tumour was consistent with granular cell tumour (GCT). GCT is a benign tumour that often involves the skin and subcutaneous tissue. Rarely has it been reported to involve the genitourinary system. Here we present the first reported case of GCT with renal involvement along with its MDCT imaging features. The authors also present a review of the literature along with a review of typical MDCT imaging features of enhancing renal masses with emphasis given to renal cell carcinoma and its varying subtypes.