Primary renal sarcomas are very rare. We report a case of renal leiomyosarcoma with 36 months follow-up. Neither ultrasonography, computed tomography nor magnetic resonance imaging are able to differentiate between leiomyosarcoma and renal cell carcinoma. Radical nephrectomy and adrenalectomy was curative. Diagnosis was established on histology and immunohistochemistry. There were no metastases. Histology and later on immunohistochemistry is the only mean by which these tumours can be diagnosed. After a period of 36 months, patient is alive and well.