Neurilemomas originate from the Schwann cells of nerve sheaths. They can occur ubiquitously as benign or malign variants. Renal neurilemomas are extremely rare. Imaging does not allow a certain diagnosis. Complete tumor resectioning is the only curative therapy. Effective conservative treatment is not known. Histological criteria of renal neurilemoma are: mesenchymal, spindle cell, capsulated tumor intensely positive to S-100 antiserum. Epithelial markers do not react.