Achalasia is an esophageal motility disorder for which the pathophysiology is only partially known for the idiopathic subtype. Many factors are known to be involved with the development of secondary achalasia. Neurofibromatosis is one of the most common autosomal dominant conditions affecting the nervous system, and 25% of those with the disease have been reported to have involvement of the gastrointestinal tract. Three cases of association between neurofibromatosis and achalasia have been reported in the literature so far. Laparoscopic Heller myotomy is the gold standard surgical treatment for patients with achalasia, whereas esophagectomy is reserved for the more advanced stages of disease. There have been reports of successful long-term results in patients with stage IV achalasia treated with Heller myotomy. We report the fourth case in the literature of association between achalasia and neurofibromatosis; it is the first time that such an association is successfully treated with laparoscopic Heller myotomy.