Primary familial cardiomyopathy, once exclusively associated with hypertrophic disorders, is now recognized to occur in a dilated or congestive form. In some instances, characteristic myocellular inclusions of varying morphologies have been identified. Nemaline rods are inclusions which typically have been linked with a rather benign and nonprogressive congenital myopathy. We report finding myocellular inclusions consistent with nemaline rods in two brother who died with congestive cardiomyopathy. Although there was no history or clinical evidence of a myopathy, characteristic nemaline rod inclusions were also identified in the skeletal muscle of one sibling.