Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital disorder characterized by multiple venous malformations in the skin, soft tissues and gastrointestinal (GI) tract. Patients with this syndrome may develop iron deficiency anemia from chronic GI hemorrhage and require lifelong treatment with iron and blood transfusions. In addition, GI lesions can be treated using endoscopic techniques such as sclerotherapy, band ligation, coagulation, and polypectomy. We describe here the anesthetic management of a 12-year-old male patient with BRBNS for endoscopic polypectomy under general anesthesia.