Pemphigus vulgaris (PV) is an immune-mediated disease manifested as flaccid bullae and extensive erosions of the skin and mucosa. The incidence of PV before or during pregnancy is uncommon, with only 36 cases were published in current English articles between 1966 and 2014. Management of PV includes immunosuppressive, intravenous immunoglobulins, or biologic agents. Currently, there is no established guideline for the management of PV in pregnancy. A 22-year-old pregnant woman at 13 weeks of gestation complained of skin erosions and blisters that worsen for 2 months before hospital admission. Dermatological examination revealed painful erosions and blisters with positive Nikolsky signs on the entire body and numerous oral ulcers accompanied by dysphagia and difficulties in eating. Histopathological examination showed suprabasal clefts filled with acantholytic cells extending to hair follicle units consistent with pemphigus vulgaris. She had been managed with systemic corticosteroid under obstetrician supervision, antibiotic, and supportive care. Her condition was significantly improved over the first month of medical treatment. Pemphigus vulgaris in pregnancy is a challenging case due to the course of the disease itself and the complications of long-term use of the therapeutic agents to both maternal and fetal. Without proper treatment, PV may be fatal due to dehydration and secondary bacterial infections. Patients with PV in pregnancy should be monitored intensively with a multidisciplinary approach to obtain a good outcome for the maternal and the baby © 2022 Pakistan Association of Dermatologists. All rights reserved.