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Homocystinuria is a disorder of methionine metabolism, leading to an abnormal accumulation of homocysteine and its metabolites in blood and urine. It is most often diagnosed in childhood and has variable expressions. We report a case of a 7-year-old male child with diminished vision since birth diagnosed as bilateral congenital cataract and superomedial subluxation of lens posted for cataract excision under general anesthesia. The key points of perioperative management included prevention of hypoglycemia, optimal hydration, prevention of thromboembolic episodes, and total intravenous anesthesia.