Introduction: Chiari malformations are a group of congenital anomalies which involve the hindbrain and the cervical spinal canal.
Case presentation: Here, we describe a patient who presented with acute diplopia and gait unsteadiness which was first deigned with Chiari malformation type-1. However due to progression of the ataxia the full neurologic evaluation was considered which established the diagnosis of spinocerebellar ataxia type 3 (Machado-Joseph-Disease).
Conclusion: We aim to highlight the importance of careful examination in order to avoid misdiagnosis of even rare diseases.
Keywords: Ataxia; Case Reports; Chiari Malformation; Spinocerebellar Atrophy.
© 2020 Tehran University of Medical Sciences.