Highlights. Autonomic dysfunction syndrome is an urgent issue in modern medicine, however, it should be an exceptional diagnosis, established after a comprehensive diagnostic study, as many diseases can often appear as this syndrome. The presented case describes the patient with a combined pathology of Kimmerle`s anomaly and Chiari malformation, who was diagnosed with autonomic dysfunction long time ago.Abstract. Autonomic dysfunction syndrome (ADS) is a topical issue of modern medicine. Differential diagnosis of this syndrome is often a difficult task for a practicing physician, because an organic disease of various etiologies can be hidden under the guise of autonomic dysfunction. The article analyzes a clinical case of a patient with a combined pathology of Kimmerle`s and Arnold-Chiari anomalies, which were not diagnosed for a long time due to the fact that clinical symptoms (syncope and presyncope conditions, dizziness, pain in the occipital region, aggravated by physical exertion and emotional stress, rises in blood pressure up to 160 mm Hg) are identical to those in ADS. After prolonged treatment of ADS without any positive effect, the child was sent to the Research Clinical Institute of Pediatrics and Pediatric Surgery named after academician Yuri Veltischev. Having received surgical treatment, the patient had no complaints for 6 months. The presented clinical case confirms that the diagnosis of ADS should be an exceptional diagnosis, established only after a complex and comprehensive examination.