Introduction: Hyperhomocysteinemia is a risk factor for thrombosis and ischemia involving different organs of body, but its prevalence data from the Indian subcontinent is scarce. It usually presents with stroke or ischemic heart disease or portal and superior mesenteric vein thrombosis or deep vein thrombosis in lower limbs. Case report: We report a case of hyperhomocysteinemia in a 24 years old chronic alcoholic male, who presented with superior mesenteric thrombosis and stroke. The patient underwent segmental resection of small intestine and temporary ileostomy. He was treated with anticoagulants, vitamin supplementation and supportive care for stroke. He has recovered from hemiparesis, is doing well without any further thrombotic episodes during two years follow up. Conclusion: Young patients presenting with acute thrombotic episodes without any embolic source or atherosclerotic disease should be evaluated for hyperhomocysteinemia.