Rett syndrome is a disorder of early brain development which is clinically characterized by arrested neuro-development. We found one such 5.5 years old girl whose physical and mental development was normal up to 17 months of age, followed by regression. She had lost her already acquired purposeful hand movements, appearance of stereotyped hand movements, along with development of epilepsy. To our knowledge such case is being reported for the first time from Bangladesh. The purpose of this case report is to increase awareness of this syndrome among physicians specially paediatricians, thereby aiding early diagnosis and treatment.