53-year-old male complained of right flank discomfort. Scans revealed solid lesion with necrotic content in the left kidney, and radical nephrectomy with ipsilateral para-aortic lymph node dissection was performed. Pathology diagnosis was a clear cell renal carcinoma. The experts recommends a close routine follow up together with routine opthalmoscopy in order to detect retinal angiomas that can be an affect of VHL gene mutation.