BackgroundTo our knowledge, there is no previous report in the literature of non-traumatic neglected complete cervical spine dislocation characterized by anterior spondyloptosis of C4, extreme head drop, and irreducible cervicothoracic kyphosis.Case presentationWe report the case of a 33-year-old Caucasian man with a 17-year history of severe immune polymyositis and regular physiotherapy who presented with severe non-reducible kyphosis of the cervicothoracic junction and progressive tetraparesia for several weeks after a physiotherapy session. Radiographs, computed tomography, and magnetic resonance imaging revealed a complete dislocation at the C4–C5 level, with C4 spondyloptosis, kyphotic angulation, spinal cord compression, and severe myelopathy. Due to recent worsening of neurological symptoms, an invasive treatment strategy was indicated. The patient’s neurological status and spinal deformity greatly complicated the anesthetic and surgical management, which was planned after extensive multidisciplinary discussion and relied on close collaboration between the orthopedic surgeon and the anesthetist. Regarding anesthesia, difficult airway access was expected due to severe cervical angulation, limited mouth opening, and thyromental distance, with high risk of difficult ventilation and intubation. Patient management was further complicated by a theoretical risk of neurogenic shock, motor and sensory deterioration, instability due to position changes during surgery, and postoperative respiratory failure. Regarding surgery, a multistage approach was carefully planned. After a failed attempt at closed reduction, a three-stage surgical procedure was performed to reduce displacement and stabilize the spine, resulting in correct spinal realignment and fixation. Progressive complete neurological recovery was observed.ConclusionThis case illustrates the successful management of a critical situation based on a multidisciplinary collaboration involving radiologists, anesthesiologists, and spine surgeons.