Spontaneous midbrain hemorrhage is a unique brain-stem vascular lesion. Sixty-six cases have been reported in the literature, to which we add seven. Of the total of 73 patients reviewed, most presented acutely (66%), but subacute (22%) and chronic onsets (12%) also occurred. Neuro-ophthalmologic abnormalities were found in 88% of patients. Some alteration of consciousness and headache occurred in most patients. Hemiparesis, hemisensory loss, and ataxia were less frequent manifestations. Vascular malformations were suspected or proved in 37% of patients, hypertension was present in 21%, and a bleeding diathesis was noted in 5%. No underlying cause was apparent in 37%. Most patients improved with supportive care only. At follow-up, 23% of patients had no neurologic deficits. In 51% of patients, minor neurologic deficits persisted, related chiefly to cranial nerves III and IV. Moderate deficits persisted in 16% of patients, and 5% of patients died.