Vulvar lesions are clinically challenging for physicians because the differential diagnosis may include many entities. Vulvar edema is one of the most frequent symptoms and is normally attributed to a local cause. Herein, we present a case report of vulvar Crohn disease (VCD) in a 9-year-old girl, in which skin lesions preceded the systemic gastrointestinal symptoms. Both clinical features and histopathological findings guided us to an early Crohn disease (CD) diagnosis. Dermatologists often have the opportunity to detect systemic diseases at early stages. A good knowledge of the CD skin manifestations could lead to an early CD diagnosis, especially in children. The suspicion of CD in those cases in which cutaneous involvement precedes digestive symptoms is crucial to prevent future psychological and physical consequences.