The objective of this study was to describe the clinical signs, neurologic examination findings, diagnostic imaging results, and pathologic diagnosis of ossifying fibroma in the cervical vertebra of a dog. A 3-year-old spayed female Pembroke Welsh corgi dog exhibited severe cervical pain and left-sided postural reaction deficits. Magnetic resonance imaging (MRI) revealed a lobulated contrast enhancing mass associated with the C6 cervical vertebra. Due to the lack of response to pain medications, humane euthanasia was elected, and histopathologic evaluation of the mass revealed a fibro-osseous lesion most consistent with an ossifying fibroma. This neoplasm is most commonly associated with the mandible of young horses and has not been previously reported in vertebrae in veterinary medicine. Key clinical message: This case is the first report of a fibro-osseous lesion most consistent with an ossifying fibroma affecting a vertebra in veterinary medicine.