Myopathy has been found to develop spontaneously in 100% of SJL/J mice between 6 and 8 months of age. Extent of muscular involvement and mouse strength were quantified in SJL/J mice and Balb/c control mice 2-16 months old. Muscle from young SJL/J mice exhibited histopathological abnormalities and occasional inflammatory infiltrate. By 6 months, 78% of SJL/J mice had developed active myopathy. By 8 months, all SJL/J mice examined had active disease with a mean of 12.9% of muscle fibers affected. Replacement of muscle fibers by fat and/or collagen began at 10 months and was pronounced by 14 months. Significant decreases in strength scores (total body pulling force) at 6 months and 10 months of age reflected the onset of active myopathy and the onset of muscle degeneration, respectively. The spontaneous onset and 100% incidence of myopathy in the SJL/J mouse line should provide a useful model for idiopathic myopathy.