Hereditary hemorrhagic telangiectasia(HHT) is an underdiagnosed autosomal dominant disorder characterized by telangiectasia and arteriovenous malformations. The presentation is variable and can be life threatening regardless of age, gender or ethnicity. After epistaxis, gastrointestinal(GI) bleeding is the most common presentation and can pose a diagnostic and therapeutic challenge. Case report: An 80 year old man with history of essential tremors, mild epistaxis and alcohol use was admitted for massive GI bleeding that required intensive care. He had been in excellent health all his life and hospitalized once for acute alcoholic hepatitis. The epistaxis never required medical attention. He did not use NSAIDs, anticoagulation or anti-platelets medications. Six first degree relatives have had epistaxis. He appeared pale but well nourished. Careful examination did not reveal any vascular lesion in the region of the lips, tongue, palate, conjunctiva, chest, back, fingertips, palms, soles or nails. The rest of the physical exam was unremarkable except for black tarry stools. The Hgb was 5g/dl(12.6–17.8), MCV 93.3(80.6–103.7), MCH 26.8(26.1–34.1) and platelets 231(155–371). The AST 27(0–47U/L), ALT 15(0–45U/L), AP 112(30–115U/L), total bilirubin 0.6mg/dl, albumin 2.9(2.6–5.2g/dl), PT14.3sec (11.8–14.3), PTT28.6sec(22.1–38.3) and closure time of 84 sec (81–153). An EGD showed over 100 flat cherry red vascular ectasias of variable size with central blood vessels and an emanating feathery look. They were friable to light touch, and covered 50–70% of the stomach mucosa. No esophageal or gastric varices were seen. Several courses of Argon plasma coagulation(APC) were directed to the largest and most friable lesions. Colonoscopy did not reveal telangectasias. A CT scan with IV contrast did not show AV malformation, portal hypertension or cirrhosis. The patient improved with aggressive resuscitation and APC treatments. Conclusion: We hope to contribute with the ongoing efforts to increase physician awareness by reporting this unusual case. The presentation is atypical due to its very late onset, lack of external visible telangiectasia and lesions almost entirely limited to the stomach. In this case, endoscopic findings became a cornerstone in the definite diagnosis of HHT as defined by The Scientific Advisory Board of the HHT Foundation International.