Although intellectual impairment is common in patients with myotonic dystrophy, this aspect of the disease has received relatively little research attention. We examined 41 patients with myotonic dystrophy using objective neuropsychological procedures and magnetic resonance imaging. Ten patients (24%) had severe and generalized intellectual dysfunction, while lesser or no cognitive impairment characterized the remaining patients. Degree of intellectual impairment was not related to neuromuscular status or sex. Patients with severe intellectual disturbance had significantly earlier onset of both myotonia and weakness and were more likely to inherit the disease from their mother. Magnetic resonance imaging findings indicated that while degree of cerebral atrophy was not related to severity of intellectual impairment, skull thickness, focal white matter lesions, and anterior temporal lobe abnormalities were significantly more common in patients with severely disturbed intellect. This study reports a number of previously unreported cerebral magnetic resonance imaging findings associated with intellectual impairment in myotonic dystrophy, but the etiology of these changes awaits neuropathologic examination.