RESUMO
Lowe syndrome [LS] is an x-linked recessive disorder of unknown etiology resulting in ocular, cerebral and renal disorder. It is X linked recessive disorder, caused by a defective gene on Chromosome number Xq 26. Males are more affected than females. Clinical features are bilateral congenital cataract [100%], Glaucoma, Infantile hypotonia, gross motor developmental delay, reduced or absent deep tendon reflexes, muscle wasting, moderate to severe mental retardation, frequent high pitched scream seizures, growth failure, Rickets, Joint hyper mobility and renal manifestations. Prognosis is poor for normal life style. There are developmental delays, visual problems and progressive mental retardation. The patient dies in the first decade of complication of Fanconi syndrome if treatment is not provided. Lifespan can be extended with supportive therapy. A case of LS with dental management, although not ideal is presented. Mentally retarded patients needs regular dental visits to reduce avoidable dental pain