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Yedikule Göğüs Hastalıkları ve Göğüs Cerrahisi Merkezi, 3. Cerrahi Kliniği, İstanbul The primary pneumoblastoma is a rare malignant tumor and 130 cases have been reported in the English literature so far. Twelve year old boy was misdiagnosed and treated as a lung tuberculosis for 1.5 years. The axial CT scan of chest showed atelectasis of the lingular segments. The bronchoscopy revealed a mass lesion possibly an adenoma located at the proximal end of left upper lobe bronchus. Following thoracotomy, invasion of the subcarinal and tracheobronchial lymph nodes were detected and left upper lobectomy with bronchoplasty were carried out. The histopathologic diagnosis was pneumoblastoma. Chemotherapy and radiotherapy were also performed to the patient. The patient was found symptom free and well in second year, postperatively. Keywords :