Pleuropulmonary Blastoma (PPB) is a very rare, highly aggressive and malignant tumor that originates from either the lungs or pleura . It has poor prognosis and mainly occurs in children less than five or six years with three different subtypes. We report a case of a 2 year old boy presenting with cough, fever and difficulty of breathing. Chest Xray showed opacified right lung highly suspicious of a mass. CT scan (contrast) showed a large hypodense mass on the right hemithorax. Complete surgical resection of the mass was done which is the cornerstone of PBB management followed by neoadjuvant chemotherapy.