Neuropsychiatric manifestations in patients with SLE, known as neuropsychiatric SLE (NPSLE), are not uncommon clinically.[1] However, the main manifestation of the peripheral nervous system (PNS) in patients with SLE and its comorbidities, such as rare myositis, is relatively infrequent.[2] In this report, we describe multiple neurological manifestations, particularly involving the cranial nerves, in a female patient with SLE and anti-nuclear matrix protein 2 (NXP2)-positive myositis. The gene discussed is MORC3; the disease is myositis disease.