Abstract: The US Food and Drug Administration (FDA) approved 59 new molecular entities in 2018, which sets an unprecedented record. Among the list, 39 drugs receive approvals for the first time globally, and 34 products are orphan drugs. These products offer new treatment options for some diseases like acquired immune deficiency syndrome (AIDS) and migraines, and some even represent the very first options for certain indications like primary hemophagocytic lymphohistiocytosis and blastic plasmacytoid dendritic cell neoplasm. In this review, we will summarize the R & D history, clinical results and market projections of these new drugs, and provide comments accordingly.