Crimean Congo hemorrhagic fever (CCHF) is a hemorrhagic fever caused by a viral agent. Among Asian countries, Iran is an endemic region for CCHF. Reports about this infection in Iran show that adults are more affected by this disease than children. However, rural children, especially boys, are at a higher risk of CCHF than other children due to contact with livestock and their secretions. In areas with higher prevalence, it is necessary to consider the diagnosis of this disease in children who mention the symptoms of fever, petechiae and purpura, ecchymosis, decreased level of consciousness, and a history of contact with animals. Since there is no definitive treatment for this disease, timely diagnosis and hospitalization of the patient is the only way to increase the probability of survival in severe cases. The present case report presents a fatal case of definitive CCHF in a six-year-old boy with confirmed CCHF by PCR