Polycythemia Vera (PV) is a rare disorder in children and adolescents. Data on clinical and biological features as well as on treatment modalities are sparse. The V617F mutation of JAK2 has been described recently and is found in almost 90% of adult patients with PV. This mutation allows now a reliable and early diagnosis. Therapeutic management is based on phlebotomy and cytoreductive therapy. In young adults and children, interferon alpha is theoretically superior as it is effective and there is no risk of inducing leukemia. We studied the JAK2 (V617F) mutation in a sample of 200 MPD patients. Only one case of pediatric polycythemia Vera has been detected in a 17-year-old girl with the V617F JAK2 mutation.