Rhabdomyosarcoma (RMS) is the most common type of soft tissue sarcoma in children. There are two main types of RMS: embryonal and alveolar (having a worse prognosis). The treatment for childhood RMS is based on surgery, chemotherapy and radiation. In spite of very intensive therapy, 1/3 of patients suffer a relapse. The case report presents a child with facial embryonal RMS with an atypical central nervous system relapse; this, despite a comprehensive diagnostic process, was diagnosed during the autopsy. NOWOTWORY J Oncol 2016; 66, 4: 322-325