Eosinophilic gastrointestinal diseases are a group of pathological entities characterized by an eosinophilic infiltration of the gastrointestinal tract without known cause. Eosinophilic esophagitis (EE) is one of these conditions, which has three diagnostic criteria: symptoms of esophageal dysfunction, mucosal infiltration of 15 eosinophils per high-power field (HPF) microscope and no response to high-dose proton pump inhibitors (PPIs). A 51-year-old patient attended to the emergency department claiming to suffer from heartburn, dysphagia, regurgitation and chest pain. He was treated with high doses of PPIs without success and the condition progressed to food impaction. An esophagogram, which showed esophageal motor dysfunction in the middle and lower part of the esophagus, was performed, and pathologic examination of an esophageal biopsy taken by endoscopy revealed histopathological changes suggestive of gastroesophageal reflux disease. An esophageal manometry was carried out and it reported diffuse esophageal spasms and a hypertensive inferior esophageal sphincter with incomplete relaxation. A Heller cardiomyotomy was performed and the biopsy specimen revealed fibrosis and eosinophilic infiltration of the muscular layer. Symptoms ceased for 6 months after which symptoms reappeared. The patient was diagnosed with EE and treated with budesonide orally 12 mg/day with complete remission of all symptoms. In this peculiar case, the eosinophilic infiltration was in the muscular layer of the esophagus, not in the mucosa; therefore, it did not meet the diagnostic criteria of EE nor its differential diagnoses. Nevertheless, treatment with oral steroids was in fact effective. J Med Cases. 2014;5(12):621-626 doi: http://dx.doi.org/10.14740/jmc1942w