Abstract Direct acting oral anticoagulants (DOACs) have guaranteed safety and convenience of use in anticoagulated patients. DOACs only require monitoring of hepato–renal function. But how to handle a patient with persistently elevated INR (International Normalized Ratio) in the absence of anticoagulant therapy? We present the case of a 77 years old woman with hypertension and hypothyroidism. The patient was admitted to our division for high frequency atrial fibrillation with initial heart failure. The patient gradually improved with rate control therapy (metoprolol and digoxin) and a few diuretic therapy. The echocardiogram didn’t showed anything significant. Laboratory tests showed a persistently elevated INR, even after she achieved clinical stability. Liver funcion tests and abdomen echography were normal. Patient’s CHA2DS2–VASc risk score was 3 with indication to anticoagulant therapy. We opted for rate control therapy because, in the absence of a diagnosis, there was uncertainty as to start or not anticoagulation therapy, with the doubt whether the persistently high INR corresponded to real anticoagulation. We didn‘t want to expose the patient to bleeding risks by initiating a DOAC. Blood tests showed mild to moderate factor X deficiency with prolonged dRVVT (diluited Russel Viper Venom time) but normal aPTT (activated partial thromboplastin time). Factor X (FX) deficiency is a rare hereditary bleeding disorder characterized by decreased antigen and/or activity of FX leading to mild to severe bleeding symptoms. Severe congenital form manifests itself early while heterozygous patients are often asymptomatic. Indeed, no previous literature data are reported about use of DOACs in this kind of patient. In mild to moderate FX deficiency there are no known contraindications for the use of anticoagulant therapy. According to the hematologist we started low–dose dabigatran, the only DOAC that acts by directly inhibiting thrombin. Regular 6 months follow–up. In conclusion this case was particularly challenging because of the lack of indications about how to manage these patients. The complexity of patients in real–life goes beyond the guidelines, which must therefore be adapted to the individual case