Pleuropulmonary blastoma (PPB) is a very rare intrathoracic malignancy in childhood. It is an aggressive embryonal or blastemic neoplasm usually occurring in children younger than five years of age. PPB is treated with aggressive multimodal therapies consisting of surgery and chemotherapy. We present a case with PPB type II successfully treated with complete surgical resection following neoadjuvant chemotherapy. She has been free of disease for 33 months of follow-up. Complete surgical resection of the tumor at the time of diagnosis is the cornerstone of PPB management, but in the majority of patients, initial surgery is incomplete because a large tumor may involve vital structures. For this reason, patients with initially unresectable tumors should be treated with neoadjuvant chemotherapy to reduce the lesion to the point that it becomes resectable.