Claude’s syndrome is a distinctive brainstem syndrome characterized by ipsilateral third cranial nerve palsy with contralateral hemiataxia and is due to an intrinsic or extrinsic lesion in the midbrain. We report a case of Claude’s syndrome caused by cerebrovascular accident due to Hyperhomocysteinemia. A 21year young male was admitted to our hospital with complaints of ataxia, right ptosis and diplopia. Brain magnetic resonance imaging (MRI) showed an acute non haemorrhagic infarct in midbrain on right side with hypoplastic left transverse sinus. Hyperhomocysteinemia was diagnosed by assessing the Serum Homocysteine level. The patient was treated with Intravenous Mannitol, Aspirin and Multivitamin supplements. The most common cause of Claudes’s syndrome is cerebrovascular disease and malignancy. Stroke is one of the most common causes of morbidity and mortality. Hyperhomocysteinemia has emerged as an important independent risk factor.