The purpose of this study was to describe the clinical, computed tomographic (CT), and pathologic findings of large cell neuroendocrine carcinoma (LCNEC) of the lung. CT and pathologic findings as well as clinical features of surgically proven LCNEC of the lung were reviewed retrospectively in 11 consecutive patients (eight men and three women; mean age, 63 years; range, 44-77 years). Chest CT showed peripheral mass or nodule (n = 8) and central mass with distal atelectasis (n = 3). Six tumors were accompanied by mediastinal (n = 3) and hilar (n = 3) lymph node enlargement at CT. On pathologic examination, all resected tumors showed necrosis of variable extent (mean: 38%, range; 10-70%). The areas of intrinsic lipoid pneumonia and tumor emboli in two patients appeared at CT as areas of ground-glass opacity surrounding the tumor. Mediastinal nodal metastases were seen in three (27%) patients. Pathologic staging of 11 patients was IB in six, IIA in one, IIB in one, IIIA in two, and IIIB in one. Follow-up data showed extrathoracic metastases in four patients at mean follow-up period of 15 months. One patient died of distant metastasis 5 months after the surgery. CT findings of LCNEC of the lung are nonspecific and similar to those of other non-small cell lung cancers and extrathoracic metastasis is seen in approximately one third of the patients with follow-up study.