Achalasia of the esophagus is a relatively rare problem in children, but it may be the cause of severe lung disease, growth retardation and respiratory death in young infants. Surgical esophago-cardio-myotomy remains the treatment of choice, and this article details 25 years of experience with 35 children with achalasia of the esophagus and their late post-operative follow-up. The occurrence of achalasia in the first six months of life, the existence of a familial factor, the prevalent possible association with genetic diseases (familial dysautonomia, glucocorticoid insufficiency, Rozycki syndrome) suggest that achalasia in childhood may in certain cases represent a congenital problem, somewhat different from the adult form, which is considered to be an acquired disease.