This paper presents a family with sick sinus syndrome, spanning three generations and with an autosomal dominant trait. The proband was affected by atrial fibrillation with a slow ventricular rhythm that required a permanent pacemaker. Her three sons were affected with a sinus node dysfunction and one daughter died suddenly at the age of 32 years. A pacemaker was implanted in the proband and her two sons with symptoms related to bradycardia. One of her sons with the pacemaker died of a cerebrovascular accident several months later. We report a family with sick sinus syndrome requiring the implantation of a pacemaker with a review of the literature.