ARVC is a hereditary cardiac disease observed in high prevalence in the Boxer breed. The disease is characterized by monomorphic ventricular tachycardia, syncope, and sudden death. Histologically, the disease is diagnosed by the infiltration of fat and fibrous tissue beginning in the area of the right ventricular outflow tract and progressing, from epicardium to endocardium, throughout the ventricle. Medical management of ARVC is the only treatment option at this time. Generally, long-term prognosis is poor. This case report shall discuss the clinical presentation of ARVC in the boxer dog, and relate these signs to the genetic mutations, histopathological, and molecular abnormalities found in cardiac tissues affected with ARVC.