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Hyperhomocysteinemia is due to genetic and acquired changes in the metabolism of homocysteine. It is associated with an increased risk for vascular occlusive disease and thrombosis. Because methylene tetrahydrofolate reductase abnormality is a genetic disorder and hyperhomocysteinemia along with pregnancy in women further aggravates the risk of thrombosis; therefore, these patients undergo antepartum anticoagulant treatment with low molecular weight heparin. These patients pose a unique challenge to anesthetist, when it comes to choosing a type of anesthesia. Neuraxial anesthesia techniques may be relatively contraindicated in anticoagulated patients and nitrous oxide may exacerbate the condition, by inhibiting the conversion of homocysteine to methionine. In this study, we intend to discuss the anesthetic implications and management of a pregnant patient with hyperhomocysteinemia undergoing cesarean section.