The Literature is reviewed with reference to primary leiomyosarcoma of the kidney, and an additional case of this highly malignant tumor is reported.

• 摘要肾脏平滑肌肉瘤为肾脏肉瘤中最常见的肿瘤，好发于四十岁，为一非常恶性且肿瘤本身能长到相当大的体积。