Background. The optimal treatment for management of esophageal achalasia in pregnancy is controversial. Little information exists about pregnancy outcome after successful myotomy. Case. Achalasia in pregnancy was diagnosed when a patient presented with pneumomediastinum from microrupture of the overdistended esophagus. An attempt at surgical correction failed due to the development of aspiration pneumonia with general anesthesia. Conservative medical therapy was undertaken, but fetal growth restriction developed. The patient underwent interval surgical correction, but subsequent pregnancy 6 months later was complicated by acute diaphragmatic hernia necessitating preterm delivery. Conclusion. Prior to surgery in pregnancy, emptying the dilated esophagus via nasoesophageal tube suctioning maybe warranted to avoid aspiration. Women, despite having undergone successful myotomy, should be counseled on the risks of pregnancy and to avoid pregnancy for at least 1 year thereafter.