Heller myotomy is recognized as the optimal treatment for achalasia. However, treatment of the markedly dilated esophagus has been debated in the literature. Although esophagectomy has been the standard treatment historically, several studies have examined successful treatment of achalasia with laparoscopic Heller myotomy in the setting of a markedly dilated esophagus (>6 cm). Patients with extreme megaesophagus (>10 cm) are often treated with esophagectomy. We report the successful treatment of 4 patients with extreme megaesophagus with laparoscopic Heller myotomy. Three of the 4 patients also had Toupet fundoplication. The average esophageal diameter was 11.2 cm (10 to 12 cm). In addition to severe dysphagia, all patients had preoperative signs, symptoms, and radiographic evidence of esophageal compression of their heart and lungs. All patients reported relief of their preoperative symptoms. Esophagectomy has not been required to maintain adequate clinical results in any of our patients. We conclude that laparoscopic Heller myotomy is an appropriate alternative to esophagectomy and can be offered to patients with extreme megaesophagus.