Neurofibroma of kidney is an extremely rare tumor. To our knowledge, only five such cases have been reported worldwide till date. Here, we report a solitary neurofibroma of right kidney in a 54-year-old woman. Radiological investigations (ultrasonography and computed tomography) detected a solid mass in the upper pole of right kidney and clinicoradiologically renal cell carcinoma was suspected. A radical nephrectomy was performed under diagnosis of cancer. Microscopically, tumor consisted of benign spindle-shaped cells accompanied by fibrous tissue. Differential diagnoses of neurofibroma and solitary fibrous tumor (SFT) were made on morphological features. Immunohistochemically, the tumor cells were positive for S-100 protein, but negative for CD34 and CD99. Thus, a diagnosis of neurofibroma was established.