Pleuropulmonary blastoma is a rare and aggressive malignant tumour that affects young children. A case of pleuropulmonary blastoma has been presented in a 9-year-old male child who was initially diagnosed and treated as pulmonary tuberculosis. Computed tomography of chest revealed a large heterogeneously enhancing mass with calcification in left hemithorax and left pleural effusion. The mass was seen to invade mediastinum and left hemidiaphragm. Thoracotomy revealed unresectable tumour involving both lobes of left lung with invasion of mediastinum and left hemidiaphragm. Biopsy confirmed type II pleuropulmonary blastoma and the child was treated with chemotherapy and radiotherapy.