Tuberous sclerosis is an autosomal dominant disease characterized with benign hamartomatosis lesions which may involve all organs but most commonly involve skin, brain, heart, eye and kidney. Renal angiomyolipoma (AML) is a benign tumor including mature fat cells, flat muscle tissue and thick walled blood vessels in different ratios. Angiomyolipomas are the most common urological involvement of tuberous sclerosis complex (TS). Although angiomyolipomas may be asymptomatic, they can be diagnosed with palpable mass, pain and hematuria complaint and may cause morbidity and mortality by massive bleeding. We wanted to share our giant renal angiomyolipoma case which was diagnosed with a palpable mass and caused a massive bleeding and was treated surgically and review the current literature with the findings.