ABSTRACT
Arnold-Chiari malformation type 1 is a congenital disease characterized by herniation of the cerebellar tonsils through the foramen magnum. Most common clinical symptom is pain, including occipital headache and neck pain, upper limb pain exacerbated by physical activity or valsalva maneuvers. Various otoneurological manifestations also occur in patients with the disease, which has usually associated with dizziness, vomiting, dysphagia, poor hand coordination, unsteady gait, numbness. Patients with Arnold-Chiari malformation may develop vertigo after spending some time with their head inclined on their trunk. Positional and down-beating nystagmus are common forms of nystagmus in them. We experienced a 12-year-old female who presented complaining of vertigo related to changes in head position which was initially misdiagnosed as a benign paroxysmal positional vertigo.