Pleuropulmonary blastoma (PPB) is a rare dysontogenetic and aggressive intrathoracic malignant pulmonary neoplasm of early childhood. We present a case of pleuropulmonary blastoma in 2.5 year old female child who was referred to our hospital due to right side pleural effusion and right hydropneumothorax. Chest scan showed a large lytic lesion occupying most of the right hemithorax. Right posterolateral thoracotomy with excision of cyst was performed; the tumor was arising from the lower lobe and invading the chest wall. Histologically the lesion was pleuropulmonary blastoma type 2 with primitive looking round to oval blastematous cells and sarcomatous differentiation. Prognosis of this type of tumor is grave with 5 year survival is 42%.