Introduction: Diagnosis of amyotrophic lateral sclerosis (ALS) requires of the exclusion of several pathologies of the cranio cervical junction that mimic ALS. The importance of such exclusion is in fact sustained by the prognostic implications that ALS has.
Case Report: We report the case of a 58-year-old woman with bulbar onset sporadic ALS associated with Chiari type I malformation on the MRI of the craniocervical junction.