Pulmonary blastoma is a rare form of lung cancer with a reported incidence of 0.25-0.55 of primary pulmonary cancers. Pleuropulmonary blastoma (PPB) is a common finding in children while it is rarely found in adults. In the past few years, the incidence of a second primary tumour has increased to 3.5% followed by third primary tumour at 0.5% and fourth tumour at 0.3%. The clinical significance of diagnosing and distinguishing a secondary primary tumour is often challenging. As per our knowledge, this is the first case of metachronous PPB in an adult patient previously diagnosed with endometrial cancer.