Study Design: A case report and review of previous literature are presented. Objective and Background: The objective of this manuscript was to report a case of destructive osteoblastoma with secondary aneurysmal bone cyst of cervical vertebra in a child, and discuss the pathogenesis of this disease. The combination of osteoblastoma and aneurysmal bone cyst in the cervical spine is rare in primary bone neoplasm. To the authors’ knowledge, only one case in a child has been reported. Method: Plain X-rays, technetium bone scanning, CT scan and MRI indicated an expansile, partially sclerotic lesion of the C4 involving the body of vertebra and appendix. The lesion was excised through anterior and posterior approach. Results: After operation the tumor was removed completely. There has been no sign of tumor recurrence or clinical or radiologic sign of instability in the follow-up investigations. Conclusions: We report a rare case of destructive osteoblastoma with Secondary aneurysmal bone cyst of cervical vertebra in a child, a full investigation indicated that complete resection of the tumor can prevent recurrence and malignant transformation. Long-term follow-up is needed to declare a lifelong cure of the disease.