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Background and aims Vulval Crohn's disease (VCD) is a rare extra-intestinal cutaneous manifestation of Crohn's disease. VCD is often unrecognized and misdiagnosed and can be difficult to treat. The aim of the study was to describe the clinical presentation, associated features, and response to treatment modalities in patients with VCD. Methods A prospective review of patients with VCD who were referred to our specialist dermatology clinic from 2003 to 2017 was performed. Data on age at diagnosis, presenting signs, associated features, and response to treatment were collected. Results Thirty-one patients with a median age of 40 years (range 13-66 years) were identified. VCD manifested most frequently with vulval edema (77%), ulceration (35%), and fissures (39%). Eighty-one percent had active gastrointestinal disease. Oral involvement and pyoderma gangrenosum were present in 13% and 19%, respectively. Topical therapy was effective in mild VCD. Tumor necrosis factor-α inhibitors were the most effective second-line therapy, with 53% achieving complete clinical remission. Additionally, we have found compression garments and shorts to be useful adjuncts in treating vulval edema. Conclusions Vulval Crohn's disease is a diagnostic and therapeutic challenge. We recommend consideration of tumor necrosis factor-α inhibitors at an early stage.