Chondromyxoid fibroma of bone (CMF) is a rare benign primary bone neoplasm accounting for less than 0.5% of all primary bone neoplasms. The spine is an uncommon site for this tumour, with forty-two cases reported in the modern English literature. They have clinical features similar to CMF arising at other sites. Local recurrence is well documented.We report an incidentally discovered lytic lesion of the C2 vertebra. The patient underwent stereotactic CT guided trans-oral curettage of the lesion with iliac bone graft and anterior fusion of C2 and C3. Microscopic examination of the surgical specimen demonstrated CMF. This is the second reported case of this rare tumour in this location.We review the literature and the unique radiological and pathological features and management of spinal CMF. Local recurrence of spinal CMF and its management is also discussed in light of the five previously reported cases of local spinal recurrence. (C) 2005 Elsevier Ltd. All rights reserved.