Childhood disintegrative disorder is a rare pervasive disorder. It was first reported by Theodor Heller under the name of dementia infantilis in 1908. Severe regression in social and communication skills occurs after apparently normal development in the first 3-4 years of life. Estimated prevalence of Childhood disintegrative disorder is around 1 per 100.000 children. Although gender distribution of the disorder is not exactly known, male seems to outnumber female cases. The prognosis is usually very poor and most individuals are left with severe developmental delay especially in language and social skills. The nature of the illness and its relation with other pervasive developmental disorders has not been solved yet. Neurological disorders and other medical conditions are sometimes associated with this disorder. The present report describes a case who developed childhood disintegrative disorder after approximately three years of normal development. The childs behavioral problems reportedly began at that age following the death of his mother. No neurological abnormalities were found as a result of EEG and serebral MRI. During the treatment symptoms have been controlled by antipsychotic drugs. [Anadolu Psikiyatri Derg 2013; 14(2.000): 174-176]