Objective : Craniovertebral junction (CVJ) consists of the occipital bone that surrounds the foramen magnum, the atlas and the axis vertebrae. The mortality and morbidity is high for irreducible CVJ lesion with cervico-medullary compression. In a clinical retrospective study, the authors reviewed clinical and radiographic results of occipitocervical fusion using a various methods in 32 patients with CVJ instability. Methods : Thirty-two CVJ lesions (18 male and 14 female) were treated in our department for 12 years. Instability resulted from trauma (14 cases), rheumatoid arthritis (8 cases), assimilation of atlas (4 cases), tumor (2 cases), basilar invagination (2 cases) and miscellaneous (2 cases). Thirty-two patients were internally fixed with 7 anterior and posterior decompression with occipitocervical fusion, 15 posterior decompression and occipitocervical fusion with wire-rod, 5 C1-2 transarticular screw fixation, and 5 C1 lateral mass-C2 transpedicular screw. Outcome (mean follow-up period, 38 months) was based on clinical and radiographic review. The clinical outcome was assessed by Japanese Orthopedic Association (JOA) score. Results : Nine neurologically intact patients remained same after surgery. Among 23 patients with cervical myelopathy, clinical improvement was noted in 18 cases (78.3%). One patient died 2 months after the surgery because of pneumonia and sepsis. Fusion was achieved in 27 patients (93%) at last follow-up. No patient developed evidence of new, recurrent, or progressive instability. Conclusion : The authors conclude that early occipitocervical fusion to be recommended in case of reducible CVJ lesion and the appropriate decompression and occipitocervical fusion are recommended in case of irreducible craniovertebral junction lesion.