Myxomas are uncommon soft-tissue neoplasms, which are extremely rare in the kidney with ten cases reported in the literature.

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’.

In this report, the clinical and histopathological findings, differential diagnosis and possible histogenesis of a case of myxoma that was originated from the left kidney of a 27 year-old female patient is presented.