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BACKGROUND: Primary renal primitive neuroectodermal tumor/extraskeletal Ewing's sarcoma (PNET/EES) is a very rare renal tumor. CASE REPORT: We report a case of primary renal PNET/EES of the kidney in an adult patient and describe its computed tomography and magnetic resonance imaging findings, including diffusion weighted images along with a review of the current medical literature. CONCLUSIONS: Although very rare, a relatively large renal mass which shows very infiltrative growth pattern on CT and MR imaging and striking diffusion restriction should raise the suspicion of a renal primitive neuroectodermal tumor, in a young adult.