| Introduction  Ewing's sarcoma is the second most common bone tumor  of childhood and adolescence, although it can also occur in soft tissues. It is  an aggressive neoplasm with improved survival thanks to medical advances, reaching  survival in 70% of cases; however, an atypical presentation is related to a  poor prognosis. Objective  To carry out a case report with an atypical  presentation in an adolescent with clinical and radiological characteristics of  rapid-onset skeletal metastases (within 4 weeks after diagnosis) and to review  the current literature on this pathology. Clinical case  This is a case of a 14-year-old adolescent with no  significant pathological history with Ewing's sarcoma, who debuted with a  rapidly evolving lesion in the frontal area, being an atypical presentation,  which is related to decreased survival. Conclusions  Ewing's sarcoma is an aggressive malignant neoplasm  that mainly affects the skeletal system in children and young adults; there are  several factors related to the management and prognosis of these patients. An  unusual presentation is associated with a poor prognosis. Keywords: Sarcoma,  Ewing, Extraosseous Ewing sarcoma, Adolescent |