The authors report the clinical signs and histological findings in 62 patients with ocular myopathies divided into two groups: (a) ocular myopathies with mitochondrial abnormalities, themselves divided into pure (19 cases) or progressive (15 cases) ophthalmoplegia, and multisystemic myopathies (14 cases); (2) oculopharyngeal myopathies (15 cases) in elderly subjects, with constant intranuclear tubulofilamentous inclusions. Among myopathies with mitochondrial abnormalities, ocular forms with a favourable prognosis coexisted with earlier and more severe multisystemic lesions.