Inflammation of the vulva can present as a manifestation of a localized problem or as part of a systemic disorder. Granulomatous vulvitis is a rare inflammatory condition that has histologic similarities to Crohn disease. Clinically, it presents with painless, chronic relapsing erythema, and edema, typically affecting the genital area. We report a case of extensive granulomatous vulvitis in a patient with no gastrointestinal evidence of Crohn disease. We describe difficulties with diagnosis and limitations in the treatment of isolated vulval granulomatous disease and aim to promote earlier recognition of the disease.