Abstract
We report the case of a 6 years old male child presenting with deep vein thrombosis and intra-cardiac thrombi. Despite anticoagulation treatment, the thrombi progressed leading to cardiovascular failure requiring a surgical approach. The initial clinical presentation, unusual progression and exclusion of differential diagnoses, guided to the suspicion of Behçet's disease. The patient developed new symptoms and responded to anti-inflammatory and immunosuppressive treatments supporting this diagnosis. Behçet's syndrome is a systemic inflammatory disease whose incidence in the paediatric population and aetiology are still unknown. Furthermore there are no evidenced-based treatment guidelines.