Objectives: To report two patients with watermelon stomach associated with systemic sclerosis (SSc) and review the literature on that subject. Methods: We describe the clinical presentation, course and outcome of our two patients and reviewed the medical literature registered in the MedLine PubMed database from 1966 to 1999 by using the key words watermelon stomach, gastric antral vascular ectasia, systemic sclerosis, scleroderma.Results: The two patients presented with microcytic hypochromic anemia. Esophagogastroscopy showed multiple linear vascular malformations in the antrum compatible with watermelon stomach. They responded to Nd-Yag laser therapy with resolution of the lesions and improvement of the anemia. Patient 1 had a history of diffuse SSc while patient 2 developed limited SSc 2 years after the diagnosis of watermelon stomach. A literature review disclosed 16 documented case reports of watermelon stomach associated with SSc, 14 of whom were women. In most cases, watermelon stomach occurred in patients with established SSc but in some it antedated it by several years. In many cases, other autoimmune syndromes such as hypothyroidism, primary biliary cirrhosis, and Sjögren's syndrome were present. The presenting symptom was iron deficiency anemia, which in 11 cases was severe enough to require blood transfusions. Nine patients were successfully treated with several transendoscopic treatments, four required surgical intervention, and in three treatment was not specified. Conclusion: Although watermelon stomach is a rare syndrome, it is recognized as a cause of persistent bleeding in patients with SSc. Awareness of this condition may increase its recognition and treatment. Semin Arthritis Rheum 30:127-131. Copyright © 2000 by W.B. Saunders Company