Gastric epithelioid leiomyomatous tumors can present in three patterns; intramural, endogastric and exogastric based upon the relation to the gastric muscular wall. Patients may experience no symptoms from this tumor or may complain of symptoms suggesting peptic ulcer disease. Upper gastrointestinal tract series are the most reliable diagnostic tests but important additional information can be obtained by ultrasound and abdominal computerized tomography. The overwhelming majority of epithelioid leiomyomatous tumors are found in the gastric antrum. Round or polygonal cells are seen histologically. Often these cells have a clear zone that surrounds the nuclei. The origin of these tumors is debated. Less than one-third of these tumors metastasize. Complete surgical excision can often achieve cure. The exogastric lesions are the ones most readily cured by resection. Prognosis also correlates with clinical parameters including size of tumor, duration of symptoms and weight loss. Adjuvant therapy has not proved beneficial.