Background: Xanthogranuloma of the stomach is an extremely rare disease, and only a few cases have been reported. Case presentation: A 71-years-old male was referred to our hospital because of subepithelial tumor of the stomach on health medical examination. On endoscopic examination, there was an about 2. 0cm sized subepithelial lesion at greater curvature of prepyloric antrum. Endoscopic ultrasound revealed that 2cm sized heterogenous mass from third layer of stomach wall, including anechoic, cystic degeneration, suggestive of bleeding or necrosis. In the cystic portion, the mural nodule was detected, suggestive of malignant potential of submucosal mass. During the laparoscopic wedge resection, the submucosal lesion was shrinked and after the resection, brownish fi uidwas squeezed from the cystic portion. Histopathologic examination of the resected specimen demonstrated submucosal nodular xanthogranuloma with central fi brinoid necrosis and cystic change. Conclusions: We report an extremely rare case of gastric submucosal xanthogranuloma which was initially malignant subepithelial lesion because of internal echogeneity with cystic degenetation. Further accumulation and investigation of this entity is necessary.