Although cutaneous Crohn's disease is well recognized in adults, in children it is extremely rare. Our purpose was to describe five children with cutaneous Crohn's disease and to review the literature. The medical records of five children with cutaneous Crohn's disease were retrospectively reviewed for clinical features and laboratory data. An extensive review of the literature was conducted. Five children, one boy and four girls, 6 to 12 years of age at onset, had cutaneous manifestations of Crohn's disease. Three had genital swelling, and the other 2 had buttock abscesses. Most were seen before the diagnosis of gastrointestinal Crohn's disease was made. There have been 80 cases of cutaneous Crohn's disease described, including our series. Only 14 were in children. Two thirds of children with cutaneous Crohn's disease had genital involvement compared with about half of the adult cases. Sixteen of the 80 patients had cutaneous lesions without preceding gastrointestinal Crohn's disease. Of these, approximately 70% had genital lesions. Although Crohn's disease is common in children, cutaneous manifestations are rarely a presenting sign. However, when cutaneous Crohn's disease is present in children, it commonly precedes the gastrointestinal disease.