Neurofibromatosis or von Recklinghausen's disease is the most common inherited syndrome predisposing to neoplasia. Carcinosarcoma is a rare malignant mixed tumor of the lung. Association of carcinosarcoma of lung with Neurofibromatosis-1 is not common. A 57-year-old man presented with history of fever, cough, hemoptysis, breathlessness, weight loss, chest pain. Multiple cutaneous neurofibromas and café au lait spots were revealed by physical examination. A homogeneous opacity was found in the right middle and right upper zone on posterior-anterior chest radiography. A 8x8x7 cm mass that had irregular borders in right upper posterior and apical segment was seen on contrast enhanced chest computed tomography. On bronchoscopy, the lumen of right upper apical segment was obstructed with vegetating tumoral lesion. The biopsy taken from this region was diagnosed as carcinosarcoma by histopathological and immunohistochemical examination.A rare case with carcinosarcoma of the lung and Neurofibromatosis-1 was reported.