PURPOSE: Primary chronic osteomyelitis (PCO) of the jaw is a rare, nonsuppurative, chronic inflammation of an unknown cause. The disease is not age specific. So far, only case reports of this disease with an onset in childhood or adolescence have been described. PATIENTS AND METHODS: Review of the patient data of our department from the past 30 years revealed 11 patients with an early onset of PCO in childhood and during puberty. RESULTS: Demographic data, clinical course, radiologic and histologic examinations, and treatment modalities are described and compared with the literature with special emphasis on the somewhat confusing terminology used for this pathology. CONCLUSION: We present a group of 11 patients with an early onset of PCO of the mandible, which, to our knowledge, is the largest described series to date. The series shows the complexity of this disease in a young patient population, demanding a careful evaluation of each case before initiation of therapy.