Askin tumor is an uncommon malignant neoplasm in the thoracopulmonary region mainly occurring in children and adolescents. Four young patients with histologically proven Askin tumors were treated in our hospital. In all patients, chest computed tomography imaging demonstrated a chest wall mass with or without destruction of ribs. All patients underwent radical mass resection and postoperative chemotherapy. By the time this article was completed, two of the patients had died with local chest wall recurrences. Here we focus on the imaging features, differential diagnosis, pathology and prognosis of this rare disease.