Cutaneous Crohn disease refers to granulomatous skin manifestations not contiguous with gastrointestinal affected areas. It is a very rare condition, especially in children, and is easily misdiagnosed when characteristic gastrointestinal symptoms are absent. We report a 10-year-old Caucasian girl with a 6-month history of erythematous, firm tumescence of the left labium majus pudendi and moist vegetations circumscribing the anal ostium. Histologic analysis of skin biopsy specimens from both types of lesions showed a characteristic granulomatous noncaseating infiltrate throughout the dermis. Endoscopic examination and a colon biopsy specimen showed chronic granulomatous inflammation consistent with Crohn disease. Treatment with prednisolone 20 mg daily, metronidazole 250 mg three times daily, topical corticosteroids, and mupirocin ointment produced marked improvement of the vulvar edema, whereas the perianal lesion had a relapsing course. Early recognition of extra-intestinal manifestations of Crohn disease, which are extremely rare in children, may be difficult. Coexistence of contiguous and noncontiguous lesions, representing the very first signs of the disease, are further peculiarities in our patient.