Angiocentric lymphoma, which is the malignant counterpart of angiocentric immunoproliferative lesions, comprises a rare group of non-Hodgkin's lymphomas of T-cell origin. It is characterized by marked invasion and destruction of small vessels by lymphomatoid cells, predominantly in the lungs. The prognosis is poor and many patients die within several months. To our knowledge primary involvement of the genitourinary tract has not been previously reported. We report a case of a solitary primary angiocentric lymphoma of the kidney in a patient with the acquired immunodeficiency syndrome. Therapy consisted of nephrectomy without adjuvant treatment. Histological characteristics as well as diagnostic and therapeutic options are discussed.