A four-year-old boy presented with a solitary bone cyst in the odontoid process and body of the axis. Plain radiographs showed a radiolucent lesion with extreme thinning of the cortex and MRI demonstrated a high signal intensity in the interlesional matrix. The cystic component extended into the body of the axis through a defect in the epiphyseal plate. At operation, the cavity of the cyst was found to contain serosanguineous fluid, and histological examination showed that it was lined by a thin layer of connective tissue. The cyst may have originated from a defect in the epiphyseal plate.