Abstract Alopecia areata (AA) describes nonscarring, generally patchy, hair loss that can affect the entire scalp [alopecia totalis (AT)] and body [alopecia universalis (AU)]. AA is considered to be an acquired autoimmune condition. However, cases of congenital AA (CAA) have been reported. This study aimed to summarize and analyse data from existing CAA reports. No funding was obtained for this research. Following the PRISMA guidelines, PubMed and Web of Science were searched. Records reporting cases of AA present at birth were included. Twelve patients were identified, with a female predominance (n = 7; 58%). Three were premature (25%). Alopecic pattern at birth was AA in eight cases (67%), AT in two (17%) and AU in two (17%). A positive family history of AA was reported in five cases (42%). The AA diagnosis was clinical in eight cases (67%) and biopsy-proven in four (33%). Nine patients received/reported treatment (75%). Of those treated with topical corticosteroid (TCS) alone (n = 3/9; 33%), two demonstrated total regrowth (67%), while one experienced development into AU (33%). Two patients were treated with TCS and a topical vasodilator (TV), minoxidil (22%). One experienced full regrowth, while the other demonstrated partial regrowth, until the TCS was discontinued (i.e. the TV was used alone), and AT resulted. One patient was treated with TCS and oral antihistamine (11%), and one was treated with placebo lotion (11%). Both experienced partial regrowth. Two patients were treated with oral pulse steroid, a topical immunomodulator and TV, but no follow-up was provided (22%). Limitations of this review include small sample sizes and the possibility of missed cases due to a lack of well-established terminology and under-reporting. Additionally, not all cases were biopsy-proven. Biopsy is not necessary for AA diagnosis but is helpful in confirming unique (e.g. congenital) presentations of the condition. Given that AA is typically considered an acquired autoimmune condition, congenital cases call our understanding of AA into question. However, a positive family history in five cases suggests that genetic factors are involved in the development of CAA, as with acquired AA. Additionally, the female predominance of CAA is in keeping with acquired AA. Finally, given that TCSs yielded partial or total hair regrowth in five of six patients who received them (83%), TCSs are a reasonable first-line treatment option for CAA. In conclusion, CAA should be considered as a differential diagnosis of alopecia in the newborn, and increased CAA awareness should result in an increased number of patients receiving proper treatment.