Lung cancer is the most preventable cancer worldwide but has a poor prognosis. Recent advances in the study of lung cancer stem cell (CSC) populations has led to a growing recognition of the central importance of cells with stem cell-like properties in lung tumorigenesis. High number of CD133+ cells is associated with the maintenance, metastasis and drug-resistance of lung cancer. CD133 serves as a stemness biomarker for CD133+ CSCs, which have been found in lung cancer tissues. This article reviews the major studies supporting the existence and importance of CD133+ CSCs in the maintenance, metastasis and drug resistance of lung cancer. Continued research in the field of CD133+ CSCs biology is vital, as ongoing efforts promise to yield new prognostic and therapeutic targets.