Malignant peripheral nerve sheath tumor (MPNST) originates from Schwann cells or pluripotent perineural cells, which is an extremely rare tumor that occurs in the kidney. We report a case of MPNST in transplanted kidney that was diagnosed in a 32-year-old man with a history of kidney transplantation. Contrast-enhanced MRI and F-FDG PET/CT features of MPNST are described, which can accurately discriminate MPNST from infection and benign tumors. These features could potentially provide valuable information to distinguish it from other renal malignancies. F-FDG PET/CT may be a useful tool for the primary diagnosis and the initial staging of MPNST.