To explore the clinical manifestations and MRI features in serum NMO-IgG-positive patients with neuromyelitis optica spectrum disorders (NMOSDs). Methods: Thirty-four serum NMO ? IgG-positive patients with NMOSDs were recruited to the study. Retrospective analyses of clinical material, laboratory statistics, and imaging data were performed. Results: Among the 34 patients with NMOSDs, the male to female ratio was 1∶16, and the average onset age was (44.65±5.27) years old. Symptoms during the first attack were abnormal sensation (52.94%), limb weakness (44.12%), diminished vision (29.41%), low-grade fever and somnolence (8.82% ), intractable hiccups and vomiting (8.82% ), diplopia (2.94% ), and stumble (2.94% ). Clinical syndromes included myelitis (82.35% ), optic neuritis (50.00% ), area postrema syndrome (14.70% ), diencephalic syndrome (8.82% ), cerebral syndrome with NMOSD-typical brain lesions (17.65% ), and other brainstem syndromes (14.70% ). The number of attacks was 1~8, with an average of (2.41±0.63). Twenty-four patients (70.59% ) experienced a relapsing-remitting disease course, and 10 patients experienced a monophasic disease course. MRI showed occurrence rates of intracranial lesions to be 35.29% in the brainstem, 17.65% in the cerebral hemisphere, 8.82% in the diencephalon, and 8.82% in the optic chiasma. Spinal cord lesions were most frequently located in the cervicothoracic cord (50.00%) and were also commonly in the thoracic cord (17.65%) and cervical cord (14.70% ). Twenty-four patients (70.59% ) showed long spinal cord lesions (≥3 vertebral segments), and among these, the lesions of 11 patients (32.35% ) extended over 10 vertebral segments. Serum NMO ? IgG-positive rate was 100% , and cerebrospinal fluid (CSF) NMO ? IgG-positive rate was 29.41% . Antinuclear antibody (ANA) positive rate was 38.24%; thyroid peroxidase antibody increase rate was 32.35%; thyroglobulin antibody increase rate was 17.65%. Conclusion: Serum NMO-IgG positivity with NMOSDs is commonly seen in middle-aged females. Abnormal sensation, limb weakness, or visual loss is usually one of the primary symptoms during onset. Myelitis and optic neuritis are common clinical symdromes. Most patients experience a relapsing-remitting course of disease. The focal lesion is usually located in the cervicothoracic cord, spanning ≥3 spinal segments. Additionally, abnormal immune parameters are frequently seen.