Lesch-Nyhan disease is a rare X-linked recessive genetic disorder caused by a deficiency of hypoxanthine-guanine phosphoribosyltransferase enzyme. The patients with Lesch-Nyhan Syndrome have several problems such as positioning and difficult intravenous cannulation because of spasticitiy. Also these patients have risk for bradycardia, pulmonary aspiration, convulsion and sudden death. Anesthetic implications of the patients with Lesch- Nyhan Syndrome are not well described. Here we report our anesthesia experience in a 4 years-old child with Lesch-Nyhan syndrome, who underwent cataract surgery. Patients with Lesch-Nyhan syndrome should be carefully evaluated in the preoperative period especially to determine the difficulties in venous access and positioning. These patients should be closely monitored for the potential of pulmonary aspiration, and sudden death postoperatively.

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