A 3-year-old male presented with an episode of respiratory distress that was superimposed on a 2½ years history of recurrent chest infections. The chest radiograph and a contrast enhanced CT scan revealed a cystic cavity in the left upper lobe with an endobronchial soft tissue mass in left bronchus. Left bronchotomy and removal of the endobronchial soft tissue mass and left upper lobectomy were performed. The histology of the specimen revealed a pleuropulmonary blastoma (PPB) type II. The presentation, diagnosis, and management of this uncommon case of PPB with intrabronchial extension are described. Pediatr Blood Cancer 2010;54:1026–1028 © 2010 Wiley-Liss, Inc.