Abstract. Leiomyomas are rare, benign submucosal tumors originating from smooth muscle cells. The clinical presentation is usually asymptomatic, with excellent prognosis. We herein report the case of a 68-year-old female with a solitary leiomyoma found during esophagogastroduodenoscopy for hematemesis. Histopathological examination revealed smooth muscle proliferation supported by positive staining for smooth muscle myosin heavy chain and negative staining for CD117 and S-100, consistent with the marker expression pattern of leiomyoma. We report on the clinical presentation of this case, and discuss the origin, epidemiology, treatment and management of leiomyomas.