Lowe syndrome is a rare X-linked recessive multisystem disorder affecting the eyes, central nervous system, and kidney. The clinical features include congenital cataract, growth and mental retardation, areflexia, hypotonia, and renal tubular dysfunction (Fanconi's syndrome). The chronic metabolic acidosis may be the most important component affecting anesthetic management. Also, problems such as electrolyte imbalance, hypotonia, seizure, and increase of intraocular pressure should be considered during the perioperative period. We report the experience of anesthetic management for removal of corneal keloid in a patient with Lowe syndrome.