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A boy in early adolescence was diagnosed with left-sided congenital pulmonary airway malformation (CPAM) at birth and remained asymptomatic until early adolescence, when he presented with dry cough, right-sided chest pain and breathlessness on exertion. Imaging showed right-sided pleural effusion with pulmonary nodules and skeletal metastases. Thoracoscopic biopsy confirmed pleuropulmonary blastoma (PPB) of type III. Despite receiving aggressive chemotherapy, the disease progressed rapidly and the outcome was fatal.This case highlights the diagnostic challenges of advanced PPB in adolescents and the poor prognosis once the disease metastasises. It also emphasises the importance of timely surgical management of CPAM and the need to consider screening in individuals with a family history of CPAM or DICER1 mutations.