Neuromyelitis Optica (NMO) is a rare idiopathic autoimmune demyelinating disease of the central nervous system (CNS) having a relapsing course. It consists of optic neuritis, longitudinally extensive transverse myelitis (LETM) which involves 3 or more neighbouring portions of the spine and positive serology for anti-NMO IgG antibodies. NMO is often misdiagnosed as multiple sclerosis (MS). Limited literature about NMO and its association with other systemic autoimmune diseases, such as systemic lupus erythematosus (SLE) is available so far. Here, we present a 21-year girl, previously diagnosed case of SLE seven years back, who suffered attacks of transverse myelitis. She had seropositivity for anti-aquaporin-4 (anti-AQP4) receptor antibody. An accurate clinical diagnosis is important to initiate timely immunosuppressive therapy to prevent disability. Key Words: Neuromyelitis Optica, Transverse myelitis, Systemic lupus erythematosus.