Abstract
Blue rubber bleb nevus syndrome (BRBNS) is a rare vascular malformation syndrome with cutaneous and visceral lesions frequently associated with serious, even fatal bleeding and anaemia. The syndrome is rare with possible dominant inheritance due to a gene mutation mapped on the short arm of chromosome 9.
Intra-operative enteroscopy (IOE) is the best method of identification of all lesions (particularly the tiny ones) and its treatment by endoscopic electrocoagulation or surgical excision.