Neuroendocrine neoplasm of the lung consists of a wide variety of malignant entities with distinct biological behaviors, clinical presentations, and prognosis. Therefore, diagnostic tests and treatment modalities can be quite different. It is critical to recognize the aggressive types—namely, small cell lung cancer and large cell neuroendocrine lung cancer to urgently initiate workup and treatment, which can be lifesaving. On the other hand, carcinoid tumors of the lung generally have better prognosis, but occasionally, the accurately discerning atypical carcinoid and neuroendocrine carcinoma can be challenging. Treatment has advanced in the past few years, particularly for small cell lung cancer, providing new hope for patients afflicted with this aggressive malignancy.