Currently, there are no proven drugs that are FDA approved for the treatment of dermatomyositis (DM), even though multiple clinical trials are ongoing to evaluate safety and efficacy of novel therapeutics in DM. The purpose of this review is to highlight the biological plausibility, existing clinical evidence as well as completed and ongoing clinical trials for various drugs in pipeline for development for use in dermatomyositis. The drugs with the strongest evidence have been included in this review with a focus on the mechanism of their action pertaining to the disease process, clinical studies including completed and ongoing trials. With better understanding of the underlying pathophysiologic process, there are new molecular targets that have been identified that can be targeted by these novel drugs, predominantly biologic drugs. There are various drugs being evaluated in phase II/III clinical trials that hold promise in DM. At the forefront of these are immunoglobulin, Lenabasum, and Abatacept for which phase III clinical trials are ongoing. In addition, promising clinical studies are ongoing or reported for KZR-616, anti-B cell therapy, anti-interferon drugs, and Repository Corticotrophin Injection (RCI).