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Yedikule Göğüs Hastalıkları Hastanesi ve Göğüs Cerrahi Merkezi Pulmonary blastoma is a rare malignant tumor of the lungs originating from the multipotent embryonic cells of a germ layer which is mostly seen in young adults. A 24 years old male patient presented with cough, left sided chest pain, sputum production and hemoptysis. Chest radiograph revealed a lobulated soft tissue mass (9.5x7.5 cm) with air-fluid level at the periphery of left lower lobe with a wide diaphragmatic base. A lobulated, smooth vascular mass obstructing a subsegment of posterior segment of left lower lobe was seen at bronchoscopy. Bronchoscopic biopsy from the mass was reported as carcinoid tumor. Left thoracotomy with resection of left lower lobe was performed. Pathologic sections contained circular formations, cells with hyperchromatic nuclei and eosinophilic cytoplasm, forming trabeculae and cords, double layer sequence, solid masses with vesicular nuclei and faint cytoplasm; mixoid features in the stroma and focal areas of mature cartilagenous tissue leading to the diagnosis of pulmonary blastoma (fetal adenocarcinoma type). The patient refused therapy and returned with metastatic lesions one year later. He was given cyclophosphamide 750 mg/m2, vincristine 1.4 mg/m2, doxorubicine 50 mg/m2, 100 mg prednisone for 3 courses and partial response was achieved. He died 16 months after the diagnosis. Keywords :