Our objective was to examine the brain biopsies by histopathology and investigate the prognosis of patients with myelin oligodendrocyte glycoprotein antibody-associated demyelinating pseudotumor. The clinical, MRI, and histological features of two patients with myelin oligodendrocyte glycoprotein antibody-associated demyelinating pseudotumor were reviewed. Both patients were treated with steroid plus rituximab and followed up. The brain biopsies of both cases revealed T cells, macrophages, and complement-mediated demyelination, which was in accord with multiple sclerosis-like pathology. Moreover, both cases showed favorable response to steroid plus rituximab therapy. Our cases add a new variant to the myelin oligodendrocyte glycoprotein-encephalomyelitis spectrum, which favorably responds to immunotherapy.