A 56 years old woman referred to our hospital with dysphagia and epigastric soreness. Gastroendoscopy revealed huge submucosal tumor with ulceration extending from distal esophagus to lesser curvature of stomach. Subsequent computed tomography (CT) demonstrated soft tissue mass encircling distal esophagus, and 18F-FDG PET/CT demonstrated intense 18F-FDG accumulation in it. Finally this case was diagnosed as esophageal leiomyoma based on pathologic evaluation of the surgical specimen. (Nucl Med Mol Imaging 2008;42(5):410-413)