The oculo-cerebro-renal syndrome of Lowe (Lowe syndrome) is an X-linked recessive disorder involving the eyes, nervous systems, and kidneys. The clinical manifestation of this syndrome is characterized by congenital cataracts, glaucoma, seizure disorder, psychomotor growth retardation, hypotonia, renal tubular acidosis, aminoaciduria, rickets, and osteoporosis. We report a 5-year old boy underwent general anesthesia for the treatment of multiple dental carries. During intraoperative period, marked metabolic acidosis was noted and such acidosis was partially corrected by hyperventilation. We suggest that patients with Lowe's syndrome should be attention and treated to possible anesthetic hazards such as metabolic acidosis due to renal tubular dysfunction, rise of intraocular pressure in patient with glaucoma, the fragility of the bone structures due to rickets and osteoporosis.