Blastocytic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy that can involve skin, bone marrow, lymph nodes, and other organs. The disease usually occurs in older men, and probably in children. There is no standard treatment regimen at present. Recently, tagraxofusp, a drug targeting CD123, was approved for the treatment of BPDCN abroad, which is the first drug approved for BPDCN, and also the first CD123-targeted drug in the field of oncology. This article reviews the recent research progress of tagraxofusp targeting CD123 in the treatment of BPDCN.