Factor VII deficiency is a rare coagulation disorder affecting approximately 1 in 500 000 individuals. A number of those patients will develop various conditions requiring chronic anticoagulation and invasive procedures, which might be especially challenging in the context of the disease. We review available literature and present a case of a woman with a severe factor VII deficiency treated with a direct oral anticoagulant dabigatran who underwent an uncomplicated pulmonary vein isolation with pulse field ablation.