Large cell neuroendocrine carcinoma(LCNEC)is a rare malignant disease with a poor and the clinical outcome. Although no standard chemotherapy regimen has been developed, LCNEC should be treated in a manner similar to that used for small cell lung cancer because of their similar chemosensitivity and aggressive characteristics, even though LCNEC is classified as non-small cell lung cancer. A 78-year-old man was admitted with the complaint of convulsions on the left side of his body. A chest computed tomographic(CT)scan on admission demonstrated a 4 cm mass in a left lung S9. Brain magnetic resonance imaging(MRI)revealed enhanced tumors in the left occipital lobe. The brain tumor was surgically resected and histological findings were diagnosed as LCNEC. After induction of 4 courses with irinotecan and split-dose cisplatin chemotherapy, a partial response was obtained. After 4 courses, the tumor size was increased, and the chemotherapy was changed to amrubicin. The patient is alive and with no relapse.