Individuals with inherited antithrombin deficiency (IATD) have a high risk of venous thromboembolism (VTE). Most VTE are managed with direct oral anticoagulants (DOACs), but the utility of DOACs in ATD is unreported. Patients with IATD treated with DOAC were identified from our institutions’ IATD registry. We assessed patients’ characteristics, ATD type, and initial VTE characteristics, thrombosis recurrence and bleeding rates. Thirty-three patients received DOACs for 73 (38.5, 111.5) months (median (IQR)). Prior to taking DOACs, 12 (36%) patients had VTE recurrence: these occurred after anticoagulation was ceased (four), nonadherence to VKA prior to DOAC use (3), or during heparin use in pregnancy (5). There were no VTE recurrences on standard dose DOAC, except in a noncompliant patient receiving dabigatran. There was one recurrence with compliant DOAC use – a patient receiving rivaroxaban 10mg. Six (18%) patients experienced clinically-relevant bleeding, which was predominantly menorrhagia (5/6). One major bleeding event, intracranial haemorrhage, occurred in a patient receiving full-dose rivaroxaban who had refractory hypertension (0.5 events/100 patient-years). In this cohort, compliant DOAC users had an overall VTE recurrence rate of 0.5/100-patient years, whereas with low-dose DOACs the event rate was 3.5/100-patient years. In conclusion standard dose DOACs appears efficacious and relatively safe in IATD.