Because of relatively low frequency of pathological changes of the upper cervical spine in childhood and adolescence, their management is not well known. Diagnostic problems occur due to the variety of malformations possible, persisting growth plates and ligamentous laxity. Because of this laxity, conservative treatment with immobilization is advocated even in severe dislocations as long as they are reducible. Persistent dislocations, chronic atlantoaxial instability (ADI > 5 mm), dens fractures type II and existing or progressing neurological deficits all indicate surgical intervention. Internal fixation allows easier postoperative care. Complications are rare. Patients with orthopedic problems of the upper cervical spine are presented, together with their management.