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Pulmonary neuroendocrine tumors (pNETs) are normally included in trials comprising neuroendocrine tumors (NETs) of different organs of origin. Typical and atypical carcinoids are regarded as low-grade NETs and show low proliferation rates and minor responses with classic chemotherapy regimens. Somatostatin analogs were traditionally applied in patients with carcinoid syndrome and recently also demonstrated tumor response. Recently, novel agents such as sunitinib and everolimus showed improved survival in randomized studies. These trials contained only low numbers of pNETs. Another promising field of targeted therapy in low-grade NETs includes peptide receptor radionuclide therapy. High-grade pNETs include large-cell neuroendocrine carcinoma and small-cell lung cancer. We will discuss large-cell neuroendocrine carcinoma and will address small-cell lung cancer at the end of this review.