Rhabdoid tumor of the kidney (RTK) is a rare pediatric malignancy. Its imaging features by ultrasound and CT have been well described; however, to our knowledge, there are only a handful of case reports that describe the MR appearance of rhabdoid tumor. We report the MR appearance of a RTK in an 18-month-old boy, review the literature on its imaging features, and discuss the differential diagnosis of solid renal tumors in children.