Pulmonary blastoma is a rare malignant lung tumor with aggressive behavior and dismal prognosis. It is extremely rare in children aged <18 years, and little is known about its genetic alteration and pathogenesis. Although surgical resection and adjuvant chemotherapy or radiotherapy have been applied in several cases, no standard treatment guidelines with sufficient evidence have been established. In this article, we report a case of a large pulmonary blastoma in a 7-year-old girl whose initial presentation was progressive dyspnea and productive cough. We subsequently present a review on cases involving young patients as well as genetic analysis data available in the literature.