. Atrial standstill is an extremely uncommon arrhythmia that rarely appears to be familial and genetically determined. Atrial standstill has been associated with several conditions including, but not restricted to, congenital heart disease, valvular heart disease, conduction disturbances, Brugada syndrome, myocardial infarction, and amyloidosis. Only a few cases of familial clustering of atrial standstill have been reported so far. This report represents a family with atrial standstill associated with syncope, dilated cardiomyopathy, and sudden cardiac death.