Alopecia areata (AA) is a common autoimmune disease, which is characterized by non-scarring hair loss and is liable to relapse. Pediatric AA is the most severe form of AA. Compared with adult AA, it is more likely to progress into complete hair loss on the scalp (alopecia totalis, AT) or complete scalp and body hair loss (alopecia universalis, AU).Currently, only few medical therapies are available for severe pediatric AA, which are not effective. In this article, we present a case report of a 6-year-old girl with severe AA, who had been effectively treated using the oral tofacitinib therapy. Besides, no relapse was observed after dose reduction with continued therapy. We also provide a brief review of global cases in which tofacitinib treatment was applied in pediatric AA. Tofacitinib can be applied at a minimum age of 4 years, and usually leads to hair growth in the first 3 months, while significant regrowth occurs after 6 months of continued therapy.