We studied 22 patients with first stroke and infarct limited to the midbrain on MRI. We selected these patients (8%) from 281 with posterior circulation infarct admitted consecutively into a primary care center. All patients underwent a systematic protocol of investigations including MR imaging and angiography, and echocardiography. Most infarcts fitted well to arterial territories drawn in preestablished templates. Middle midbrain involvement was the most common, mainly in the paramedian territory supplied by the basilar artery. Infarct in the mesencephalic territory of the posterior cerebral artery was less common, while superior cerebellar artery territory infarct was extremely rare, and posterior choroidal artery territory infarct did not occur. The neurologic picture was dominated by eye-movement disorders. Patients with isolated upper or lower midbrain infarct had no localizing clinical findings, but patients with middle midbrain infarct had a localizing picture mainly with nuclear or fascicular third nerve palsies that commonly developed in isolation. Vertical gaze paresis, pure motor hemiparesis, four-limb ataxia from unilateral lesion, and hypesthetic ataxic hemiparesis also occurred. Contrary to a common view, cardioembolism was not a more common etiology than basilar artery stenosis or small-vessel disease.