A case of umbilical pemphigus vulgaris is reported.A 37-year-old female presented with a half-year history of repeated oral blood blisters and periumbilical itchy blisters.Dermatolog-ical examination revealed a demarcated annular irregular erosive surface covered with odorless white secretions on the umbilical area.But neither blisters nor erosions were observed in the oral mucosa.PDAI activity scores were 2 points.Pathological examination of skin lesion showed blis-ters in the basal layer and acantholytic cells in the epidermis,and lymphocytic infiltrations in the superficial dermis.Direct immunofluorescence revealed reticular deposition of IgG and C3 in the intercellular space of the spinous layer,without IgM and IgA depostion.The diagnosis was pem-phigus vulgaris.Patient was treated with intramuscular injection of Methotrexate(10 mg)once,and oral methylprednisolone tablets at a dose of 24 mg once daily,in addition to calcium supple-mentation and topical applications of Halometasone and Sodium Fusidate Ointment.After the skin lesion was improved,the patient was discharged.No new lesions occurred during 6-month phone follow-up.