Treatment of severe alopecia areata (AA) remains difficult. To assess the tolerance and efficacy of methotrexate (MTX) in the treatment of severe long-term AA, we retrospectively evaluated 22 patients with AA totalis or universalis with a mean duration of 11.0 ± 8.8 years who were treated with MTX either alone (n = 6) or associated with low doses of oral prednisone (n = 16). MTX was given at an initial weekly dosage of 15 mg (n = 3), 20 mg (n = 9), or 25 mg (n = 10). Oral prednisone was given at an initial dosage of 10 mg/d in one patient and 20 mg/d in 15 patients. In all, 14 patients (64%) achieved a total recovery including 3 of 6 patients treated by MTX alone and 11 of 16 who had received the combined treatment. Of the 14 patients who had total hair regrowth, 6 stopped MTX. In all, 3 patients maintained hair regrowth and 3 relapsed. Retreatment of these 3 patients by MTX resulted again in hair regrowth. No severe side effect was observed. Although limited by its uncontrolled character, this study shows that MTX and low doses of oral corticosteroids may be an effective and well-tolerated treatment for severe types of AA.