BACKGROUNDSjögren's syndrome (SS) is a chronic autoimmune disease that affects the exocrine glands, causing mainly symptoms such as xerophthalmia and xerostomia, besides extraglandular manifestations such as pneumopathies and neurological disorders. Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that causes progressive and irreversible motor paralysis. The purpose of this report is to present the case of a patient with SS who developed a condition suggestive of ALS. Therefore, it is questionable whether the motor neurological involvement may be secondary to SS.