The neurologic manifestations of primary Sjögren syndrome are varied and can be divided anatomically into 2 categories: peripheral neuropathies and central nervous system (CNS) conditions. Distal sensory and sensorimotor neuropathies are the most common manifestations of peripheral nerve disease in primary Sjögren syndrome. CNS manifestations associated with primary Sjögren syndrome include focal central lesions, conditions that mimic multiple sclerosis, encephalitis, aseptic meningitis, cerebellar syndromes, movement disorders, and problems with memory, cognition, and depression. The heterogeneity of neurologic manifestations in primary Sjögren syndrome complicates the approach to treatment, which should be directed toward the underlying neuropathologic mechanism.