Large cell carcinoma of the lung with a rhabdoid phenotype is very rare. We present a 55-year-old man with multiple nodules in his lung. He had an emergency operation because of abundant hemoptysis. The microscopic appearance was a large cell carcinoma with a pure rhabdoid phenotype. There were no foci of any other carcinomatous components. Tumor cells had abundant eosinophilic cytoplasmic globules and eccentric nuclei and did not adhere to each other. Histochemically, these cells were periodic acid-Schiff-negative. Immunohistochemically, vimentin and neuron-specific enolase were positive. Epithelial membrane antigen was focally and weakly positive, p53 was positive in 60% of tumoral cells, and Ki-67 (MIB-1 labeling index) was 50%. The patient died of disseminated disease 2 months after the operation.