Abstract
BACKGROUND: Rett Syndrome (RS) is a chromosome X-linked genetic neurological disorder characterized by developmental regression, particularly in relation to expressive language and use of the hands. It is also associated with profound mental retardation and almost exclusively affects females. CASE DETAILS: A four and a half year old girl reported to our dental OPD for a dental checkup. On complete examination, she was diagnosed to be suffering from Rett syndrome. Preventive therapies and proper oral hygiene instructions were explained to her mother. CONCLUSION: Early diagnosis of such disorders is extremely important along with treatment of patients' problems with love and care to prevent them from further pain and stress.