Tuberous sclerosis complex (TSC) is a genetic condition caused by variants in the TSC1 or TSC2 genes. It leads to benign tumors, known as hamartomas, in various organs such as the brain, kidneys, skin, and heart. Symptoms can include early-onset epilepsy, varying degrees of developmental disabilities, and mental health issues. Affected children may experience anything from normal cognition to severe intellectual disabilities. A multidisciplinary approach to care is essential, focusing on early detection, diagnosis, and management of seizures. Treatments using mechanistic target of rapamycin (mTOR) inhibitors can help manage epilepsy and reduce tumor burden, improving patient outcomes. This review highlights the clinical presentation, genetic basis, and the importance of genetic counseling. It also emphasizes the need for ongoing surveillance and tailored management strategies for neurological, neurodevelopmental, renal, dermatological, and pulmonary issues throughout a patient’s life.