CASE PRESENTATION: This is a case of a 2 year-old-male who presented with productive cough two months prior associated with fever and dyspnea who was initially managed as a case of pneumonia. However, condition persisted. Work up for TB was negative.Chest X-ray showed an opacity filling the right hemithorax and leading to mediastinal shift to the opposite side.Computed Tomography revealed a large mass in the right hemithorax containing solid and cystic components. He was then transferred in this institution intubated,chest examination showed asymmetry on chest expansion,subcostal retractions and vesicular breath sounds over the left lung. The right hemithorax showed dullness on percussion,decreased vocal and tactile fremitus,and absent breath sounds.Alpha Feto protein,Urine VMA were within normal limits,Serum lactate dehydrogenase was elevated. No significant change was seen on repeat chest X-ray. Repeat CT scan with contrast showed a large hypodense mass lesion on the right hemithorax.On 2D echocardiogram,the mass was compressing the right atrium,prompting emergency surgical procedure.Excision of the entire mass with frozen section was done which showed myoplasty with heterologous component possibly chondroid fat,vascular component with atypical cellular areas.Final tissue biopsy aided by immunohistochemistry staining revealed an Pleuropulmonary Blastoma,Type II because it contained both solid and cystic components.Post procedure inflammatory changes were demonstrated with serial chest X-rays which was resolved spontaneously.He was successfully extubated and was discharged improved. He recently completed the the first course of chemotherapy after discharged.