<p>Sjögren’s disease is a&#xa0;systemic autoimmune disorder, the clinical spectrum of which extends beyond classical sicca symptoms. Extraglandular manifestations, particularly neurological involvement, are common and relevant to prognosis. Both the peripheral and central nervous system may be affected, with symptoms ranging from small-fiber neuropathy and axonal or demyelinating polyneuropathies to myelitis and central nervous system vasculitis. Clinically, progressive sensorimotor deficits resulting in disability, gait impairment, neuropathic pain, and cranial nerve involvement are frequently observed. An interdisciplinary approach is required for diagnosis. In addition to medical history taking and carrying neurological examination, electrophysiological studies, cerebrospinal fluid analysis, and MRI are essential. In cases of suspected small-fiber neuropathy, skin biopsy is an important diagnostic tool. The differential diagnosis should distinguish these manifestations from isolated chronic inflammatory demyelinating polyneuropathy as well as from metabolic, toxic, or hereditary neuropathies. In patients with polyneuropathy, clinical red flags such as sensory disturbances not only in the feet but also in the hands, progressive motor involvement with paresis, and cranial nerve involvement should raise suspicion of neuro-Sjögren. A&#xa0;phenotype-oriented treatment strategy is required. Painful small-fiber neuropathy is often initially treated symptomatically but may also require immunomodulatory therapy. Early immunomodulatory therapy should be considered in patients with progressive sensorimotor neuropathy or central nervous system manifestations. Close collaboration between rheumatology and neurology is essential to improve prognosis and functional outcome.</p>