Background: We aimed to review clinical characteristics, treatment results and outcome of pediatric patients with primary pleuropulmonary neoplasms. Methods: Medical records of 14 cases diagnosed between 1972-2009 were reviewed retrospectively. Results: The male/female ratio was 5/9 and the mean age at diagnosis was 9.1 years (2-16). All but one were symptomatic, presenting with fever, coughing, dyspnea, or weight loss. One patient presented with hemoptysis, and another with digital clubbing. One mesothelioma was diagnosed incidentally. Some 8/14 patients were initially diagnosed as having pneumonia (median delay in diagnosis of 2.5 months). Diagnoses included pleuropulmonary blastoma (PPB, n=5), inflammatory pseudotumor (n=3), mesothelioma (n=2), mucoepidermoid carcinoma (MEC, n=2), and carcinoid tumor (n=2). Patients with PPB underwent surgery and received chemotherapy +/- radiotherapy. Two carcinoid tumor cases underwent surgery, one further received chemotherapy. Patients with mesothelioma were treated with chemotherapy. Inflammatory pseudotumors were all resected. Two cases with MEC received chemotherapy, one after surgery. 2/5 PPB patients survived without recurrence, 3 died; all carcinoid tumors and inflammatory pseudotumors were alive; 1/2 MEC patients was alive after 252 months, the other one was lost without disease; 1/2 mesothelioma patients was alive without disease, the other was died. For all cases, median follow-up was 30.5 months (0.6-252). Conclusions: Primary pleuropulmonary tumors are rare but clinical presentation can be varied and delay in diagnosis is common. Children with persistent coughs, recurrent pneumonia or hemoptysis should be considered as indicators for early diagnosis, very important because the prognosis of these tumors varies with histology and stage.