Summary Tumors of the atlas and axis are exceedingly uncommon lesions. A review of the literature provided 28 cases, of which two-thirds were the result of metastatic processes. Benign endoplasms in this region may cause serious sequelae resulting from impingement upon the lower brainstem, upper cervical cord, and vertebral arteries. Vigorous therapeutic endeavors should be undertaken with the aim of achieving complete amelioration of pain, reversal of neurologic impairment, and attainment of cervical stability. Although the underlying disease process in the majority of patients may be a terminal condition, improved longevity combined with the presence of disabling pain and neurologic compromise demands carefully planned treatment. Important points discussed include the following: 1. It is necessary to establish tissue diagnosis by the appropriate method. The transpharyngeal needle biopsy of Ottolenghi is a safe and dependable method for obtaining tissue from the anterior elements of C1 and C2. 2. Cord compression may result from two basic mechanisms, and it is necessary to resort to cervical myelography to distinguish between the two. 3. Flexion and extension views, cineradiography, angiography, and tomography may assist in patient evaluation. 4. Guidelines for planning the surgical approach are offered, as well as preferred exposure techniques. 5. Anterior surgery in this region is a major undertaking and should be reserved for patients with benign anterior tumors or cancer patients with a good prognosis and specific indications. 6. Tracheostomy should be carried out to avoid postoperative complications after anterior surgery. 7. Whenever possible, bone grafting should be used to establish cervical fusion. Several drawbacks to successful bone grafting exist in the case of metastatic disease. Some of these factors may be circumvented with the use of methyl methacrylate alone or in combination with a bone graft. Acrylic also allows early postoperative ambulation without the need for cumbersome external immobilization devices.