Alopecia universalis (AU) is generally considered a variant of alopecia areata (AA), in which the treatment options seldom provide satisfactory results. However, successful treatment of several cases of AA and its variants with oral Janus kinase (JAK) inhibitors have been reported recently. Here we report a 23-year-old female patient with AU successfully treated with tofacitinib, a selective JAK-3 inhibitor. The initial tofacitinib dose was 5 mg twice daily. After 2 months of treatment, partial hair regrowth was seen on the scalp and eyebrows. Thereafter, the dose was increased to 10 mg in the morning and 5 mg at night. By 6 months of the treatment, there was complete hair regrowth throughout the entire body. Our patient tolerated tofacitinib well, without any significant side effects. Tofacitinib emerges as a promising novel therapy in alopecia universalis. We believe further study is required to establish the safety and confirm the efficacy of tofacitinib treatment for alopecia universalis.