We report the case of a 10-year-old girl with an Askin Tumor who had a history of Acute Lymphoblastic Leukemia. Seven years after the diagnosis of ALL, her family detected a mass on her left chest wall. An ultrasound-guided tru-cut needle biopsy was performed and the diagnosis of a primitive neuroectodermal tumor of the chest wall (Askin Tumor) was finalized with histologic and genetic analysis. The risk of secondary cancer is three to 10 times higher in childhood cancer survivors than the general population. Patients who have several different types of cancers should be tested for hereditary cancer syndromes.