Sotos syndrome, also known as cerebral gigantism, is a rare genetic disorder characterized by distinctive facial features, overgrowth during childhood resulting in tall stature and macrocephaly (enlarged head), and developmental delays or intellectual disabilities. It was first described in 1964 by Juan Sotos, and major diagnostic criteria were established in 1994 by Cole and Hughes. The condition is primarily caused by mutations or deletions in the NSD1 gene, leading to haploinsufficiency of the Nuclear receptor Set Domain containing protein 1. This gene is responsible for regulating the growth and development of various tissues in the body. Sotos syndrome can present with a wide range of symptoms and severity, making diagnosis challenging without genetic testing. While there is no cure for Sotos syndrome, management typically involves addressing individual symptoms and providing supportive care, including early intervention services and educational support for developmental delays. Regular medical monitoring and screening for associated medical conditions are also important aspects of management.