Atlanto-axial rotatory subluxation (AARS) often presents in childhood as a stiff, painful neck with associated typical cock-robin type of torticollis. It can occur spontaneously or following minor trauma or an inflammatory process in the cervical tissues or following otorhinolaryngeal surgery. We report a case of bilateral AARS which occurred sequentially following craniotomy. To the best of our knowledge, no case of AARS after craniotomy has been reported previously and only one case in which bilateral AARS was observed has been reported to date. Failure to recognize the condition may result in a fixed rotatory subluxation, which can be difficult to treat. Awareness of this condition by surgeons involved in brain surgery should lead to its early recognition and treatment, preventing a fixed deformity occurring. We discuss the clinical presentation and management of AARS. The literature on this subject is briefly reviewed.