AbstractAlopecia areata (AA) is an autoimmune disease directed at the hair follicle. Although usually limited to patchy hair loss over the scalp (focalis), AA can present as total loss of scalp hair (totalis; AT) or as total loss of both scalp and body hair (universalis; AU). Management of AT and AU can be challenging, and although multiple treatment modalities have been explored, no therapy is currently FDA‐approved. This review focuses on the evidence for current treatment options for AT and AU. The PubMed database was searched from January 1, 2000, to September 1, 2016, for clinical trials, retrospective studies, and case reports of treatments for AT and AU. A total of 40 studies were retrieved and analyzed. Therapies studied for AT/AU included: topical immunotherapy, steroids, photodynamic therapy, immunosuppressive agents, TNFα inhibitors, and other therapies, such as sulfasalazine, bexarotene, JAK inhibitors, and simvastatin/ezetimibe. Although certain treatments showed significant hair regrowth, no treatment was completely effective. The most promising therapies with the highest quality data include diphenylcyclopropenone, squaric acid dibutylester, photodynamic therapy, steroids, and cyclosporine in combination with methylprednisolone. High‐quality randomized‐controlled trials with large sample sizes are lacking. Unified outcome guidelines are encouraged to facilitate the comparison of future studies.