Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare disorder that consists mainly of abnormal multifocal venous malformations affecting the gastrointestinal tract, skin and soft tissues. Patients with this syndrome develop Multiple distinctive skin lesions, anaemia from chronic GI bleeding, and require blood transfusion and iron supplementation lifelong. Mortality depends on the GI involvement because it is difficult to treat GI bleeding [1,2]. The incidence of this syndrome is very low, and only approximately 200 cases have been described in the literature. Isolated GI involvement in BRBNS is very minimal and common site is small intestine. We describe a previously unreported case of isolated Blue Rubber Bleb Nevus Syndrome of Esophagus.