Abstract: Blue rubber bleb naevus syndrome (BRBNS) is a rare syndrome of AV malformation which mainly involves skin, soft tissue, gastrointestinal tract and other parts of the body. Its occurrence is usually sporadic, although cases of autosomal dominant inheritance is reported. At presentation symptoms depend on the organs involved. Patients may present with acute or chronic gastrointestinal bleed and it usually seen in children and rarely in adults. We herein reported a rare case of BRBNS in 17 years old male patient who suffered from Malena for four years with past history of haemangioma excision on back. Patient was on regular blood transfusion for six months. Endoscopy revealed multiple AV malformation in gastrointestinal tract. Patient underwent laparotomy with resection of involved segments of ileum with primary end to end anastomosis. Post-operative recovery was uneventful. So here we can conclude that the definitive management of BRBNS affecting the gastrointestinal tract is excision. This article was an original case review of management of BRBNS. The clinical presentation, required investigations and management of the same.