Neurofibromatosis (NF) is a genetic disorder inherited in an autosomal dominant pattern. Subtypes I and II are the most well recognized, and among these, Type I has associated vasculopathy. Less than 3% of patients have vascular involvement, with the renal artery as the most commonly involved vessel. Dissection and rupture of aneurysms in larger arteries such as the subclavian and the aorta have been previously reported. This is the first reported case of a spontaneous coronary artery dissection in a patient with NF-I. Imperative in the percutaneous treatment of coronary artery dissections is early recognition and proactive decision making. Recognition of a possible association between NF and coronary dissections will further facilitate successful and prompt management of this otherwise rare entity.