Familial hemiplegic migraine and its associated signs and symptoms have previously been well described. The condition demonstrates autosomal dominant inheritance and has been recently assigned to a locus on chromosome 19. Previous reports of familial hemiplegic migraine have indicated that expression always occurs early in life. We describe a patient who presented with multiple episodes of reversible hemiplegia followed by headache at the age of 75. Seven other family members were found to have been affected in a similar manner. We believe this case represents a unique description of familial hemiplegic migraine presenting in later life.