Introduction: The bean syndrome, or “blue rubber bleb nevus” (BRBN) is a rare disease (about 200 reported cases) characterized by the presence of multiple cutaneous and vascular malformations of the digestive tract and is associated with difficult to control chronic intestinal hemorrhage and consequent iron deficiency anemia. Case Report: A male patient, 18 year-old, was diagnosed with BRBN at the age of 3 years. The patient had multiple visceral, muscular, and subcutaneous vascular lesions, complicated with chronic gastrointestinal bleeding. The patient was initially treated by endoscopic ablation (argon-plasma) and surgical resection of the larger lesions, with temporary clinical improvement. Due to RBC-transfusion dependence (total of 74 transfusions since diagnosis), despite oral and intra-venous iron therapy (ferric hydroxide, iron dextran, and ferric carboxymaltose), the digestive tract was evaluated by endoscopy and capsule enteroscopy, which revealed the small bowel as the main source of bleeding. Treatment with propranolol was initiated, as so various endoscopic treatments (balloon-assisted enteroscopy with thermal argon ablation and sclerosis), but only with temporary improvement. The most recent therapy (propranolol and ferric carboxymaltose) provided symptomatic improvement without significant improvement of anemia and blood loss. The use of anti-angiogenic drugs has been delayed due to concerns of possible growth adverse effects. At 18 years old, with achievement of adult height, it was the treatment with sirolimus was proposed. After 5 months of anti-angiogenic therapy, the patient showed improvement of blood loss, asthenia, and subcutaneous/muscular lesions sensitivity and size. Sirolimus was well-tolerated and anemia also resolved (recovery of 5g/dL in 5 months). Disclosure - Helder Cardoso - Advisory committee: Abbvie, Janssen; Speaking and Teaching: BMS, Bayer, Roche.