We describe a patient with Noonan syndrome (NS) presenting with ulerythema ophrygenes (UO)--an association initially suggested in a single case series of five patients by Pierini and Pierini in 1979. Recognition of the association of UO with NS by pediatric dermatologists is important because of the high incidence of cardiovascular anomalies in NS. Therefore, in infants and children presenting with UO, clinicians should maintain a high index of suspicion for NS and refer for further workup.