Isolated massive vulvar edema in pregnancy is an extremely rare entity and a cause for concern. It has been presented in the literature only as case reports. Venous congestion and relative lymphatic obstruction is the main cause of generalized edema seen during pregnancy, however when isolated vulvar edema observed, associated factors should be investigated. Isolated massive vulvar edema might be encountered during pregnancy as an unusual presentation of multiple disorders. Many factors either related to pregnancy or not might play a role in the etiology. Local trauma, vulvovaginitis, allergic reactions, preeclampsia, prolonged labour, systemic disorders (renal, hepatic, cardiac), Behcet disease, diabetes, vulvar malignancies and malnutrition should be evaluated in differential diagnosis. In the treatment of this condition conservative therapeutic measures must be in the first place. By this paper, we presented an unusual case of an acute vulvar edema observed in a twin pregnancy at 29 weeks gestation with its management and reviewed the reported cases in the literature.