Achalasia is a motility disorder of the esophagus characterized by absence of normal peristalsis and failure of relaxation of the lower esophageal sphincter. Among the most common clinical findings are vomiting and weight loss. Roentgenographically, a narrowed gastroesophageal junction and dilated esophagus are observed. This condition is uncommon in children. Here a seven-year old female patient with achalasia is reported.