Three patients with spinal primary diffuse-type tenosynovial giant cell tumor (DTGCT) received surgical treatment in our department between 2002 and 2012. All 3 patients were female and aged 23, 33, and 44 years. The mean time from symptom onset to diagnosis was 17 months (range, 5-24 months). One case involved the C1 right lateral mass and C2 vertebral body, the second involved the C1-2 left lateral masses and C2 vertebral body, and the third involved the C5-7 left lateral mass and C6 vertebral body. All patients underwent computed tomographyguided biopsy to confirm the diagnosis of tenosynovial giant cell tumor. Gross total resection was achieved in all patients, including 2 piecemeal resections and 1 en-bloc resection. The mean follow-up time was 6 years (range, 1-11 years), and there was no sign of recurrence in the patients. Seventy cases have been identified so far in the English literature. The male to female ratio is 1:1.38. The mean patient age is 38.5 ± 17.9 years. The tumor distribution includes 32 cases in the cervical spine, 14 in the thoracic spine, 22 in the lumbar spine, and 1 in the sacrococcygeal region. The recurrence rate for patients who underwent gross total resection was 7.7%, and tumor progression was observed in 66.7% of patients who underwent subtotal resection. Above all, DTGCT is a rare primary spinal neoplasm. Preoperative image-guided biopsies play an important role in the diagnosis and treatment strategy. Gross total resection is the best treatment strategy and can reduce the recurrence rate.