ABSTRACT: BACKGROUND: Perthes Syndrome (Traumatic asphyxia) is rare, which is caused by sudden compressive chest trauma and characterized by subconjunctival hemorrhage, facial edema, craniocervical cyanosis, and petechiae on the upper chest and face. CASE PRESENTATION: We present the case of a 4-year-old Caucasian girl who developed traumatic asphyxia associated with intramedullary spinal cord hemorrhage following thoracic compression. CONCLUSION: We have not found the association of Perthes syndrome with intramedullary spinal cord hemorrhage described in the medical literature. To our knowledge, the current case is the first report of Perthes Syndrome associated with intramedullary spinal cord hemorrhage.