A 27-year-old woman was admitted to our hospital with epigastric pain and pelvic mass. Magnetic resonance imaging (MRI) of the abdomen showed a well defined 15x14x7 cm tumoral mass in the left kidney. The patient underwent a left-sided nephrectomy. On microscopic examination, the tumor consisted of large amounts of myxoid material containing a few uniformly scattered spindle cells. Immunohistochemically, tumor cells stained positive for vimentin, but not for S-100 protein, epithelial membrane antigen, pancytokeratin and smooth- muscle actin. With these histopathological and immunohistochemical findings, the case was diagnosed as ‘renal myxoma’.