Background: Pityriasis rubra pilaris (PRP) is an uncommon dermatosis in children. Few long-term studies on the treatment and prognosis of PRP in children have been performed. Objective: Our purpose was to retrospectively review the clinical course and treatment of all cases of PRP in children 19 years or younger who were seen at the Mayo Clinic. Methods: The clinical courses of the 30 patients with PRP seen at the Mayo Clinic between 1975 and 1997 were reviewed. Results: The most common presenting form of PRP in children is the type III juvenile form (Griffiths' criteria). Treatment ranged from topical steroids, tar, and ultraviolet B to systemic retinoids and methotrexate. The best response was obtained with isotretinoin; 5 of 6 patients showed 90% to 100% clearing within 6 months of treatment. Follow-up information was obtained by questionnaire and was available for 83% of patients. Overall, 43% had 90% to 100% resolution of their disease, 23% had a moderate response (30%-90% improvement), and 17% had a poor response (<30% improvement). One patient reported spontaneous resolution. Seventeen percent of those who had total clearing had recurrence of PRP within 1 year. Conclusion: PRP in children is a noninherited dermatosis with no sex predilection, occurring mainly in the type III classic juvenile form. Retinoids should be considered as first-line treatment for PRP. Recurrence rate, previously thought to be rare, was about 17% in our population. (J Am Acad Dermatol 2002;47:386-9.)