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Sheri- Kashmir Institute of Medical Sciences, Department of Neurosurgery, Jammu & Kashmir, India DOI : 10.5137/1019-5149.JTN.2582-09.1 Compression at the craniovertebral junction because of tumors is not a very common entity. The commonest tumors present here are neurofibroma and meningioma. Any vertebral tumour can be present at this location. Benign bony tumors are very uncommon at this location and amongst these enchondromas are exceptionally rare. Enchondromas are rare bony tumours of chondrogenic origin. These are benign tumours with a propensity for malignant transformation. There are four histological types: osteochondromas, enchondromas, chondroblastoma and chondromyxoid fibroma. Enchondromas are often asymptomatic because of their slow growth but may have varied presentation. An enchondroma may occur as an individual tumor or as several tumors together. We here report a case of enchondroma arising from the atlas and causing myelopathy. The best treatment is complete excision which we could achieve in our case. Keywords : Enchondroma, Atlas, Chandrogenic tumour