Aneurysmal bone cysts predominantly occur in young adults and the long bones, the lumbar spine and the pelvis are mainly affected. This article presents the case of a 22-year-old woman with the very rare localization of an aneurysmal bone cyst of the atlas and an atlas fracture after a minor trauma. The initial radiological diagnosis was a suspicted aneurysmal bone cyst which was confirmed histologically. Due to the unstable fracture it was decided to carry out surgical treatment with occipitocervical stabilization in combination with a transoral bone graft. After a period of 11 months the fracture had completely healed and the implants were removed without any complications.