Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by malformations and benign neoplastic lesions of the central nervous system and various non-neural tissues. It can involve the brain, skin, kidneys, heart, and lungs. Incidence: 1/5800 to 1/10000. In 1908, Vogt first described the clinical features of TSC: a triad of head and facial angiofibroma, seizures and mental retardation. In fact, only 29% of patients have a distinct triad of symptoms, and 6% of patients do not show the triad at all. The causative loci for TSC are located in the 9q34 region (TSC1) and 16p13.3 (TSC2). The natural mutation rate is high, and the rate of episomes varies widely among families. Sporadic patients reach 50-84%. The relationship between genotype and phenotype is very complex due to the presence of a large number of ectopics. Typically, patients with TSC2 have more severe clinical symptoms. This includes the possibility of earlier onset of seizures, poorer intelligence, and higher number of nodules. The 11 main clinical features are facial angiofibromas or fibrous spots on the forehead, nontraumatic periapical finger and toenail fibromas, depigmented spots (≥3), sharkskin-like spots (connective tissue nevi), cerebral cortical nodules, subventricular nodules, subventricular giant cell astrocytomas, multiple renal vascular smooth muscle lipomas, cardiac rhabdomyosarcomas (single or multiple), lymphatic smooth muscle neoplasms, and retinal malformations of the fundus 9 secondary clinical features 9 secondary clinical features multiple randomly distributed enamel pockets, malformed colonic polyps, bone cysts, cerebral white matter radial migrations, dental fibromas, non-renal malformations, fundus pigmentosa, coffee milk spots, polycystic kidney. Definite TS: 2 major features or 1 major feature plus 2 minor features. Probable TS: 1 major feature plus 1 minor feature. Suspected TS: 1 major feature or ≥ 2 minor features.