Angiomyolipoma(AML) is one of the most common benign renal tumors. Classical AML is benign, but it can be locally invasive, extending into perirenal fat, or in rare cases, invading the renal collecting system, renal vein or inferior vena cava and right atrium. About 10% of patients clinically diagnosed with renal AML have tuberous sclerosis complex. Tuberous sclerosis complex can be diagnosed by genetic diagnosis or clinical manifestation. We report a rare case of a 35-year-old woman who was diagnosed with tuberous sclerosis complex caused by TSC2 gene mutation, which was characterized by multiple angiomyolipoma in the right kidney and extended growth to the inferior vena cava and right atrium. Intracardiac extension is often observed in the malignant tumor and only seldom seen in benign tumors. Our case reminds the rare possibility of intracardiac extension in renal AML, which may potentially result in fatal complications if not appropriately managed.