Achalasia is a motor disorder of the esophageal smooth muscle in which the lower esophageal sphincter does not relax normally with swallowing, and the esophageal body undergoes nonperistaltic contractions. The underlying abnormality is the loss of intramural neurons. Achalasia affects men and women of all ages. Dysphagia, chest pain, and regurgitation are the main symptoms. Information on the effects of achalasia on pregnancy outcome and the effects of pregnancy on the natural course of achalasia is limited. Two studies, including 30 patients altogether, and several case reports have been published. Treatment options include nitrates, calcium channel antagonists, botulinum toxin injection, pneumatic dilation, and esophagomyotomy.