Thirteen infants and children with proved gastroesophageal (GE) reflux had complaints that suggested a CNS disorder. Symptoms began in early infancy in 10 cases, but accurate diagnosis and proper treatment were not instituted in 3 cases until 3-5 yr of age. A CNS basis for their disease was suspected because they exhibited specific signs or because the importance of associated gastrointestinal (GI) and respiratory tract symptoms was not appreciated. The presenting CNS symptoms and signs included dystonia in 11, developmental retardation in 10, dysphagia in 9, seizures in 6 and extreme irritability in 10. The diagnosis of symptom-causing GE reflux is probably being missed regularly. The effects of proper medical or surgical therapy are often dramatic, and the consequences of missed diagnosis or improper treatment are potentially lethal.