The clinical and computed tomographic (CT) findings in 11 proven cases of mitochondrial cytopathy (mitochondrial myopathy, Kearns Sayre syndrome, ophthalmoplegia plus) were studied. The CT changes included focal low density lesions in the basal ganglia and white matter and atrophy which could be slight or diffuse and severe. Calcification has been described in the basal ganglia, but did not occur in our series. Serial CT showed progression of the abnormalities. The differential diagnosis is discussed.