Arnold Chiari malformation (ACM) is herniation of brain stem structures, especially cerebellar tonsils, from the foramen magnum into the cervical spinal canal. Sleep-related respiratory disorders are reported at a higher rate in this patient group than the general population, at about 60-75%. A 43-year-old male patient was diagnosed with ACM type 1.5 at the center where he applied with complaints of weakness in the extremities, speech disorder and syncope triggered by coughing, and a decompression of posterior fossa (PFD) was applied to the patient. Since 2018, the apnea attacks, which were able to be 6-7 times a night every night, last in 30 seconds, with eyes open, inactivity and bruising, have started in the patient, so in all night-time polysomnography (PSG) examination for diagnostic purpose, apnestic breathing including continuous, central and mixed type apneas in sleep and wakefulness was detected. Different modes (CPAI BPAP-S/T, ASV) were tried with the cannula placed on the tracheostomy in the PSG performed for non-invasive mechanical ventilation therapy, but none alone was effective. In the patient, whom IVAPS/AVAPS mode was tried, all abnormal breathing events, irregular apnetic breathing pattern and oxygen desaturations were effectively controlled in all positions, wakefulness and all sleep stages with pressure support, respiratory rate and tidal volume support. PFD is recommended as the first-line treatment for the treatment of obstructive or central type apneas in ACM; Information on non-invasive mechanical ventilation therapy is insufficient. In this case, we aimed to present the difficulties in the diagnosis and treatment of sleep-related respiratory disorders in a patient whom ACM diagnosed, PFD was applied but underwent permanent trachoestomy due to bilateral vocal cord paralysis.