A 56-year-old female and her 34-year-old daughter presented with a predominantly distal myopathy affecting the peroneal and calf muscles, neck flexors and hand muscles. Both patients and two other daughters had cardiac arrhythmias, three requiring the insertion of cardiac pacemakers. Skeletal muscle biopsies revealed a complex myopathic process with granular degeneration, rimmed vacuoles and eosinophilic cytoplasmic inclusions. Ultrastructurally, the inclusions were composed of electron dense granular material and filaments forming linear masses beneath the sarcolemma and rounded masses within the cytoplasm of the fibres. Immunohistochemistry revealed labelling of the inclusions for desmin, dystrophin and vimentin, but not for alpha-actinin, spectrin, utrophin or myosin heavy chains. This family shows a hereditary distal myopathy with some features in common with previously-reported cases in which biopsies showed cytoplasmic inclusion bodies containing desmin. This group of diseases is clinically and pathologically heterogeneous. In the present cases, the accumulation of cytoplasmic filaments may reflect a generalised disturbance of filamentous protein metabolism rather than a specific disorder of desmin.