The association between inherited thrombophilia and the occurrence of (recurrent) VTE has been demonstrated in the past focusing only on a few genetic defects including antithrombin (AT), protein C, protein S deficiencies and two polymorphisms, factor V Leiden (FVL) and prothrombin G20210A mutations.231Surprisingly, the vast majority of information clinicians daily use for the management of thrombophilic patients is based on the results of previous studies only dealing with thrombophilia mechanisms discovered in the second half of the last century. This evidence concerns the gene BTK and Rare hereditary thrombophilia.