BACKGROUND Clinical features of upper cervical spinal cord tumors are not clear because there have been too few published reports. The purpose of this study was to review the clinical features of these tumors. METHODS We reviewed 13 patients who underwent surgery for an upper cervical spinal cord tumor. Data regarding age, sex, duration and type of symptoms, levels, topographical locations, surgical results, and histological features were investigated retrospectively. RESULTS Of the 13 tumors, 5 were at C1/2, 7 at C2/3 and 1 at C1-3. Topographically, 9 of the 13 tumors were dumbbell shaped, and all 5 at C1/2 had this shape. The initial symptom was occipital pain in three patients, numbness or pain of the extremity in nine, and clumsiness of the upper extremity in one. The average duration from initial symptom to surgery was 14.9 months. There were no major surgical complications, but there were three cases of postoperative cervical kyphosis. These three patients underwent tumorectomy with total laminectomy of C2. Tumor recurrence was seen in three patients for reasons thought to be the same as tumors at other levels, being residual meningiomas lying ventral to the cord and extraforaminal neurinomas. CONCLUSIONS Spinal cord tumors in the upper cervical region tend to progress as dumbbell tumors. As all the neurinomas had this shape (Eden type 2 or 3) at C1/2, this anatomy might favor progression to the extradural and extraforaminal spaces.However, it also allows total removal of the tumor via a posterior approach. To maintain postoperative cervical alignment, the surgeon should select the least invasive approach to the paraspinal muscles attached to the spinous process of C2.