Prader–Willi syndrome (PWS) is a genetic disorder which occurs with a frequency of about one in 10,000–30,000 live newborns. Both males and females, and all races are equally affected. PWS is a complex disorder with multiple disabilities, and the main defect is found in the hypothalamus. Child with PWS at the age between 2 and 3 years becomes constantly hungry and if the diet is not controlled, often leads to obesity and type 2 diabetes. Because of the presence of xerostomy, people with PWS have tendency to develop cavities and periodontal disease, and early application of preventive and prophylactic measures is very important. Before invasive dental treatment, consultation with a physician who treats the underlying disease is necessary, and the application of general anesthesia is often required due to mental retardation. If the pediatric dentists become more familiar with PWS, the diagnosis and treatment of oral pathology will start earlier, which is essential to improvement of the general health and the quality of life and care for these individuals. Multidisciplinary approaches are necessary for dental management of the orofacial problems in patients with PWS.