Alopecia areata is an acquired, chronic, non-scarring hair disorder of the skin affecting 0.5-2% of the general population worldwide. Multiple mechanisms are involved in the disease, namely genetic predisposition, environmental triggers, impaired hair growth, and altered inflammatory and immune responses. Recent progress in the understanding of immune pathophysiological mechanisms has opened interesting perspectives for innovative treatment strategies. Several strategies have been tested, with debated results. However, proof of concept in humans of targeting of the Interferon (IFN)γ/Th1 pathway and of the Janus Kinase (JAK) signaling pathway has led to the development of several topical and oral JAK inhibitors in this disease with high unmet needs. Our review covers novel immune mechanisms of the disease and promising therapeutic approaches already tested in clinical trials and/or under development.