Primary rhabdoid tumor of lung is a rare histological and clinical entity. Lung tumors with rhabdoid features have been included as variants of large-cell carcinoma in the 1999 World Health Organization (WHO) classification of lung tumors. A large-cell carcinoma with a rhabdoid phenotype (LCCRP) is unusual, with only 38 cases reported till date. We report the clinical details of one such case that was treated with pneumonectomy and adjuvant chemotherapy. We also present a review of the literature. To identify relevant articles, we searched PubMed, Ovid, and IngentaConnect databases using the key words 'rhabdoid,' 'lung cancer,' and 'primary rhabdoid tumor of lung.'