Abstract: Lowe syndrome also known as oculocerebrorenal syndrome. The aetiology is related to mutation of OCRL1 (Oculocerebrorenal syndrome of Lowe)gene in the chromosome Xq261，reulting in a deficiency of OCRL1 protein or a reduction of phosphatase activity is responsibility for the disease. Lowe syndrome is a multisystem disorder characterised by anomalies affecting the eye, the nervous system and the kidney. Most of affected individuals is male, female carriers may be presence of bilateral cataract without other systemic abnormities. The common ocular disease in Lowe syndrome including bilateral congenital cataract, glaucoma, keloid, amblyopia and retinal atrophy. The mainly ocular treatments contain phacoemulsification, glaucoma filtering operation, topical medication.   (Int Rev Ophthalmol, 2014, 38:   330-333)