Exogenous lipoid pneumonia (ELP) is a chronic inflammatory reaction of the lungs resulting from the aspiration of vegetable, animal or mineral oils. Squalene, is a derivative of shark liver oil that is taken as a traditional remedy in some Asian countries, and is used widely also in cosmetics. Similar to the symptoms in most cases of oil aspiration, the symptoms of squalene-induced lipoid pneumonia are either absent or nonspecific. Hence, the disease is generally detected incidentally. Although many cases with predisposing factors have been reported, ELP with achalasia is quite rare. We report a 47-year old woman with achalasia who developed ELP after ingesting squalene. The patient was treated successfully by supportive care and surgical treatment of the achalasia.