We describe a 62-year-old woman who presented with a history of ataxia, dizziness and urinary urgency. Neurological examination disclosed a positive Romberg sign, ataxia and postural instability. A magnetic resonance imaging (MRI) scan showed Chiari type 1 malformation (CM1). Forty-eight months later, the patient was clinically improved and underwent a second MRI examination, which showed complete resolution of the Chiari 1 malformation. Spontaneous resolution of CM1 is exceptionally rare and has to be considered in the radiological and clinical management.