Introduction: CVJ anomalies are very common in Neurosurgical practice. The treatment modality & surgical technique, still remains a challenge. Objectives: To study the various congenital & Acquired CVJ anomalies, their clinical presentation, management and Post surgical functional outcome. Materials and Methods: About 50 patients of CVJ anomalies who were presented at the “Instiute of Neurosurgery, Madras Medical College, Chennai-3 were analysed by their demographic, clinical & radiological parameters, and various surgical procedures. Results: About 34 patients had congenital anomalies & 16 patients had acquired anomalies.. AAD is the commonest abnormality noted. ADI determines the postsurgical outcome. C1 C2 Stabilisation carries good results. Conclusions: Bony anomalies are more than soft tissue anomalies. Craniometric assessments are very arbitrary. Congenital anomalies are very common than that of acquired ones.

Keywords: Craniovertebral Junction Anomalies; Atlanto Dental Interval; Atlanto Axial Dislocation.