Pleuropulmonary blastoma is a malignant lung tumor affecting children. Pleuropulmonary blastoma is a rare entity having an incidence between 0.25-0.5% of all primary lung malignancies that occurs exclusively in children younger than 6 years. The tumor usually originates from pulmonary parenchyma, mediastin and pleura and especially presents as a solitary pulmonary mass in unilateral lung. In this case report, we present a 3.5-year-old boy who was admitted with fever, dyspnea, wheezing, abdominal pain, weight loss and was diagnosed as pleuropulmonary blastoma. As pleuropulmonary blastoma is rarely seen in childhood, relevant literature on pleuropulmonary blastoma is reviewed.