Claude’s syndrome caused by ventromedial midbrain lesion is characterized by ipsilateral third nerve palsy and contralateral ataxia. This syndrome is very rare and only a few cases have been reported since 1912. An 81 years old male developed sudden onset right third cranial nerve palsy and left sided ataxia. Magnetic resonance imaging (MRI) brain revealed an infarction in the right ventromedial midbrain. Although the red nucleus has often been suggested as the lesion site responsible for Claude’s syndrome, a lesion of the superior cerebellar peduncle just below and medial to the red nucleus could be responsible for this syndrome.