Pityriasis rubra pilaris (PRP) is a dermatosis of unknown origin with a limited frequency in childhood (0.2% of our hospitalized pediatric patients). During the last 20 years we have observed 31 new cases; follow-up has been conducted in 29 children. The acute self-resolving form seems to be the most frequent in children, compared to adults. No cases of hereditary PRP have been observed, and no relationship between the severity of PRP and its prognosis has been reported. Since juvenile PRP has a relatively rapid course and a spontaneous resolution (a few months), it seems unnecessary to use potentially harmful drugs. Synthetic retinoids may be employed in patients whose disease is both persistent and disabling.