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ROS1 rearranged non-small-cell lung cancer (NSCLC) is a distinct molecular subtype of NSCLC, accounting for 2% of cases. Typically, these are genomic rearrangements resulting in a fusion oncoprotein that causes unabated constitutive signaling. Patients with ROS1 rearranged NSCLC have distinct clinical and histologic profiles and show excellent outcomes with various small molecule tyrosine kinase inhibitors. For the preparation of this review, we searched the literature in databases like PubMed, Embase, and Scopus with keywords such as “ROS1”, “ROS1 crizotinib”, and “Oncogene NSCLC.” We included 72 articles. This is a narrative review of the molecular biology, clinical, and pathologic characteristics, detection methods, and management of ROS1 rearranged lung cancer.