Perianal Crohn's disease in children is a potentially debilitating condition that can precede or follow the intestinal disease component. The perianal abnormalities are varied and can include lesions of the perianal skin or anal canal, abscesses or fistulas, and malignancies. The appropriate management of these problems is predicated on a thorough evaluation of the perineum and anus as well as the remainder of the alimentary tract. Therapy usually includes a combination of antibiotics, immunomodulators, and biologic agents as well as conservative operative procedures. The surgical options are intended to safely ameliorate disease-related symptoms without compromising function or continence.