Background. Transoral approach is an advanced neurosurgical technique in the treatment of atlantoaxial dislocation with spinal cord compression. Good cooperation between the surgical and the anesthetic teams during the treatment of this pathology plays a key role in achieving a positive result. Case report. Child M, 7 years old, presented with dyspnea at rest, pain in the cervical region, lower limbs, headaches, upper limbs plegia, lower limbs paresis. Diagnosis: Osteoblastic/lithic process of C1 - C2 vertebrae with C2 fracture, with anterior and left paravertebral solid conglomerate, stenosis of the magnum foramen, compression of the medulla oblongata, signs of poststenotic myelopathy. The patient had a BMI of 16.5, severe spastic tetraplegia with an upper limb accent, cervical myelopathy and moderate hypercapnic respiratory failure, a positive bilateral Babinski sign and bilateral plantar clonus. The decision of performing a medullary decompression with spinal stabilization was taken. The monitored values were those of patient’s: BP (invasive), CVP, HR, Ps, SpO2, diuresis, ABB, ionogram, coagulogram, and the results of neuromonitoring. Ist stage of the surgery was the occipito-cervical internal segmental fixation of C0-C1-C3-C4, with open C1-C2 distension, laminectomy and TIVA (14 hours). A volume of 2400 ml of blood was lost. The IInd stage of the surgery was performed after 12 days, and consisted of C1 and C2 corpectomy, with transoral cranio-cervical prosthesis and TIVA (12 hours). The patient was discharged after 21 days from the 2nd surgery. Conclusions. An acute respiratory failure developed as a result of the postoperative polyneuropathy (the right hemithorax did not participate in the respiratory act, the left one showed only low amplitude trips). The ventilator weaning was difficult and was successful only after tracheostomy was installed and only when a combination of cholinolytic and anticholinesterasic therapies was used.