- Rett syndrome was described relatively recently. It affects only females and is characterized by progressive deterioration of neurological status and impairment of psychomotor development. Psychomotor development is normal in the first year of life, but then there is deterioration of fine motor and language skills and later still, of gross motor skills. This results in severe motor function handicap and dementia. The arrested head growth, severe psychomotor retardation and typical "washing hands" movements are typical of Rett syndrome. We describe a 3-year-old Bedouin girl with the clinical picture of the syndrome and review the world literature, stressing the difficulty in detection and diagnosis.