Arrhythmogenic Right Ventricular Dysplasia (ARVD) is a familial, degenerative myocardial disease of Boxers characterized primarily by myocyte atrophy and fatty infiltration of the right ventricular free wall, with occasional involvement of the interventricular septum and left ventricular free wall. Affected dogs can be asymptomatic with occasional ventricular premature complexes (VPCs), syncopal with ventricular arrhythmias, or exhibit overt myocardial systolic dysfunction with or without evidence of congestive heart failure (CHF). In all cases, affected individuals are at an increased risk for sudden cardiac death. Antiarrhythmics are the mainstay of medical treatment in the majority of patients, however those with CHF require additional drug therapy and dietary modification. This seminar will review a case of ARVD in a middle-aged Boxer, with attention given to cause, clinical presentation, diagnosis, and treatment of the condition, as well as insights into sophisticated screening techniques and parallels to a similar myopathy in humans.