We report the case of a 3-year-old boy with severe haemophilia A presenting with recurrent haemarthroses despite daily infusions of factor VIII delivered through a central venous access device (CVAD). Regular rinsing of the CVAD with heparin, according to a standard protocol, resulted in systemic anticoagulation, as demonstrated by prolonged thrombin time and therapeutic anti-Xa levels. The bleeding symptoms resolved after replacing heparin with a normal saline solution. This case illustrates that heparin administered to maintain CVAD patency should be used with caution in young haemophiliacs. Prolonged thrombin time should alert the physician to this possible CVAD complication.