AimsBudd‐Chiari syndrome (BCS) is managed by interventions aimed at relieving hepatic venous obstruction and anticoagulation. Despite robust data supporting the tolerability and efficacy of direct oral anticoagulants (DOACs) in patients with other venous thromboembolism, its utility in BCS is not well documented. This study aims to evaluate the efficacy and tolerability of DOACs in Primary BCS from the available literature.MethodsPublished studies that reported data on patients with BCS treated with DOACs were included.ResultsTwo retrospective studies and nine case reports met the criteria for inclusion. The combined data from these two retrospective studies include 58 patients administered DOAC and 101 patients treated with VKA/LMWH. The combined re‐stenosis or failure rates after percutaneous endovascular intervention, angioplasty, TIPS, or OLT were 17.2% for the DOAC group and 15.8% for the LMWH/VKA group. The incidence of major bleeding was 8.62% in the DOAC group and 5.94% in the LMWH/VKA group, while minor bleeding rates were 20.7% and 4.95%, respectively. Procedure‐related bleeding was 4.5% in DOAC group and 12.8% in VKA/LMWH group. Nine case reports using apixaban in 3, rivaroxaban in 5, and one with dabigatran‐ described patients tolerating the treatment well and experiencing no major adverse events.ConclusionsDOACs appear to be at least equally effective to LMWH/VKA for the anticoagulation of patients with BCS. We believe DOACs to be preferred over LMWH/VKA for the anticoagulation of patients with BCS due to the known advantages in administration, but randomized trials might be needed to answer this question.