OBJECTIVE In adults renal cell carcinoma (RCC) accounts for over 85% of all diagnosed renal cancers. A much more rare and aggressive malignant tumor of the kidney is angiosarcoma (AS) with less than 25 cases described internationally. Both RCC and AS have similar radiological appearances and thus require histological evaluation for definitive diagnosis. We present a case of renal AS in a 63-year old male who was initially radiologically diagnosed as RCC, and review the current renal AS literature. METHODS The current English literature from 1981 and onwards on renal AS was reviewed and compared to our current case. RESULTS The median age and sex of patients with renal AS at presentation was 63 years old (mean 61 years) and common in males with a left kidney predominance. Symptoms included flank pain, palpable mass, and hematuria with imaging suggestive of RCC. Hematogenous metastatic spread often occurred with median survival time of 3.5 months from time of diagnosis (mean 5.8 months). Histologically, the tumors have classical features of angiosarcoma with numerous blood-filled vascular spaces lined by plump pleomorphic endothelial cells with CD31 and CD34 staining positivity. Overall treatment was radical nephrectomy with radiation therapy for local control and metastases. The use of chemotherapy was not consistent. CONCLUSIONS Although RCC accounts for the majority of malignant renal tumors, the poor prognosis of AS and its similar radiological appearance to RCC imparts the importance of histological evaluation and the potential radiological mimicry of AS.