How to Cite This Article: Gowda VK, Nanjundappa RC, Pendharkar H, Benakappa N. Homocystinuria with Cerebral Venous Sinus Thrombosis: Excellent Recovery with Intravenous Recombinant Tissue Plasminogen Activator. Iran J Child Neurol. Summer 2017; 11(3):48-52. AbstractHyperhomocysteinemia can cause cerebral venous thrombosis. Recombinant tissue plasminogen activator is one of the treatment options for cerebral venous thrombosis in selected cases. We present here a 7-year-old boy with homocysteinuria with stroke. MRI of brain showed cerebral venous sinus thrombosis. We successfully treated with intravenous recombinant tissue plasminogen activator. He recovered completely without any complications.Recombinant tissue plasminogen activator can be considered one of the treatment options in cerebral venous thrombosis in homocystinura.References1. Fernando D. Testai, MD, PhD; Philip B. Gorelick, MD,MPH. Inherited Metabolic disorders and stroke part 2-Homocystinuria, organic acidurias, and urea cycle disorders. Arch Neurol 2010; 67 (2):148-153.2. Herrmann E, Lorenzl S, Obeid R. Review of the role of hyperhomocysteinemia and B-vitamin deficiency in neurological and psychiatric disorders-current evidence and preliminary recommendations. Fortschr Neurol Psychiatr 2007; 75: 515-527.3. Online Mendelian Inheritance in Man. Homocystinuria. http://www.ncbi.nlm.nih.gov/entrez/dispomim. cgi?id=236200. Accessed March 27, 2009.4. udd SH, Skovby F, Levy HL, Pettigrew KD, Wilcken B, Pyeritz RE, et al. The natural history of homocysteinuria due to cystathionine beta synthase deficiency. Am J Hum Genet 1985; 37: 1-31.5. Hacke W, Doonnan G, Fieschi C, Kaste M, von Kummer R, Broderick JP, et al. Association of outcome with early stroke treatment: Pooled analysis of ATLANTIS, ECASS, and NINDS rt –PA stroke trials. Lancet 2004; 363: 768-774.,6. Roach ES, Golomb MR, Adams R, Biller J, Daniels S, Deveber G. et al. Management of stroke in infants and children: a scientific statement from a special writing group of the American Heart Association Stroke Council and the Council on cardiovascular disease in young. Stroke 2008; 39:2644-2691.7. Soleau SW, Schmidt R, Stevens S, Osborn A, MacDonald JD. Extensive experience with dural sinus thrombosis. Neurosurgery 2003; 52: 534-544; discussion 542-544.8. Janjua N, Nasar A, Lynch JK, Qureshi AI. Thrombolysis for ischemic stroke in children: data from the nationwide inpatient sample. Stroke 2007; 38:1850-1854.9. Amlie-Lefond C, deVeber G, Chan AK, Benedict S, Bernard T, Carpenter J et al. Use of alteplase in childhood arterial ischaemic stroke: a multicentre, observational, cohort study. Lancet Neurol 2008; 8:530-536