The disease can involve the heart, kidneys, and lungs, and the neurological symptoms associated with tuberous sclerosis, especially seizures, are clinically difficult to treat; its hallmark cutaneous manifestations include gray leaf spots, angiofibromas, finger (toe) nail fibromas, and nail bed hemorrhage (Figures 2 and 3); more than 80% of patients have a concomitant renal tumor known as vascular smooth muscle lipoma (Figure 1), which can cause spontaneous aneurysmal hemorrhage and hemorrhagic shock due to abnormal blood vessel formation. This tumor can lead to spontaneous aneurysmal hemorrhage and hemorrhagic shock due to the formation of abnormal blood vessels. Figure 1. Vascular smooth muscle lipoma: renal cortical tumor consisting of abnormal blood vessels, smooth muscle, and fat (CT hypointense). Figure 2. Gray leaf spot showing hypopigmentation and rhombic plaques accompanying the appearance of angiofibroma, a lesional manifestation of tuberous sclerosis. Figure 3. Nail bed hemorrhage, annular nail and subxiphoid fibromas typically occur in adolescence and adulthood and are more common in the toes than in the fingers. TSC is due to mutations in the TSC1 or TSC2 genes, and the formation of TSC1 and TSC2 is responsible for the regulation of mammalian target of rapamycin protein complex 1 (mTORC1). Drugs that inhibit mTORC1 (e.g., sirolimus and everolimus) are effective in the treatment of TSC, which includes renal vascular smooth muscle lipoma, refractory epilepsy associated with brain tumors, and lymphangioleiomyomatosis.