Blue rubber bleb nevus syndrome is a rare disorder characterised by cutaneous and gastrointestinal vascular malformations. A 31-year-old man came under our observation with obscure gastrointestinal bleeding. Hemangiomas on his back and the multiple bluish vascular lesions in the distal ileum revealed by capsule endoscopy led to the diagnosis of blue rubber bleb nevus syndrome. The patient was treated with octreotide to prevent rebleeding. During the 12 months of treatment he had no gastrointestinal bleeding episodes or anaemia. Then repeat capsule endoscopy showed that the small bowel vascular lesions were unchanged. Capsule endoscopy may be a useful tool for diagnosing and monitoring the effects of therapy in patients with blue rubber bleb nevus syndrome.