Pleuropulmonaryblastoma (PPB) is a rare childhood neoplasm accounting for less than one percent of all primary malignant lung tumors of children less than six years of age. Metastasis to central nervous system (CNS), orbit, iris, bone, contralateral lung, liver, kidney, and pancreas and rarely to adrenal glands has been described. This report presents a case of pleuropulmonaryblastoma with hemorrhagic pleural effusion and metastasis to the CNS at the time of presentation.