METHODS A 38-year-old man had been suffering from circumscribed scleroderma for 12 years. Dysphagia had been diagnosed 5 years ago and for the last 2 years he had retrosternal dysphagia for solid and liquid food. His symptoms had increased markedly 6 months before presenting at our hospital and the patient had lost 15 kg of weight. 2 months ago, a percutaneous endoscopic gastrostomy (PEG) had been inserted at another hospital. METHODS The patient presented in a reduced general and nutritional state. The routine laboratory tests and tumor markers were within normal range. Endoscopy showed a moderately dilated esophagus with food remnants. It was not possible to pass the cardia without exerting pressure. Esophageal manometry and barium upper gastrointestinal series revealed the classical findings of achalasia. RESULTS An extramucosal Heller myotomy with anterior semifundoplication (Dor's procedure) was performed. The postoperative course was uneventful and the patient was able to take solid and liquid food without any dysphagia. CONCLUSIONS The association of achalasia and circumscribed scleroderma has not been described in medical publications yet. The entity could possibly be based on common autoimmune mechanisms and an analogous pathogenesis with resulting fibrosis.