METHODS A case report of a rare benign osteoblastoma successfully treated with total en bloc excision. OBJECTIVE To describe the clinical and radiographic features of an adolescent female patient with intraspinal osteoblastoma in cervicothoracic junction and the effect of surgical treatment. BACKGROUND A rare case of massive benign osteoblastoma stemming from left side lamina of C7 and extension to spinal canal from C6 to T1 resulting in spinal cord compressed severely is reported. And the excellent results achieved from surgical intervention are presented. METHODS A 13-year-old girl complained of gradually increasing pain of her lower part of the neck and left shoulder radiating to the left upper extremity, and the neurologic deficits were found in physical examination. A massive neoplasm located in intraspinal cervicothoracic junction and the spinal cord compressed severely was shown in radiographs, MRI, and CT. The neoplasm was removed totally with instrumentation performed in surgical intervention. She was followed up for 2.5 years; clinical symptoms and plain radiograph and CT films were investigated. RESULTS The specimen of the removed mass was proven osteoblastoma pathologically. Her symptoms were relieved and the neurologic deficits recovered completely. Recurrence of the tumor was not seen at the final follow-up. CONCLUSIONS A case of massive intraspinal osteoblastoma stemming from C7 lamina was successfully treated by excised grossly with instrumentation for reconstruction of stability.