Large cell neuroendocrine carcinoma (LCNEC) is part of the neuroendocrine spectrum of pulmonary tumors. This increasingly recognized tumor has been reported to have 5-year actuarial survival rates following resection that are worse than those described for other variants of non-small cell lung cancer (NSCLC). Therefore, debate has emerged regarding whether the tumors should be classified and treated as NSCLC or small-cell lung cancer. This article reviews the tumor characterization, biology, presentation and diagnosis, surgical therapy, results of therapy, and long term prognosis of patients with LCNEC.