This article focuses on a rare case of primary renal angiosarcoma in a 56-year-old man with a previous clinical history of stage III Hodgkin's lymphoma treated with supra- and subdiaphragmatic radiotherapy, splenectomy in association with vincristine-based chemotherapy and thermochemotherapy, and subsequent thymectomy. The patient was referred to the department of urology from the department of cardiology, where he had been seen for right coronary stenosis after the incidental finding on an abdominal scan of a large left renal mass. There was no family history of renal cancer. Diagnosis was high-grade angiosarcoma, extensively necrotic and hemorrhagic, involving the renal parenchyma and perirenal soft tissue. Taking into account tumor histology, grade, size and site as well as patient's age and general condition, a therapeutic program was planned comprising surgery followed by chemotherapy (epirubicin 60 mg/m² and ifosfamide 3000 mg/m²). Adjuvant radiotherapy, normally delivered to the site of surgery, was not considered necessary. Unfortunately the patient died 4 months after surgery, before chemotherapy was started. Our paper highlights the extreme rarity and aggressiveness of renal sarcoma, its poor prognosis, and the fact that there is no one, accepted approach to its treatment.