The production of autoantibodies against myelin oligodendrocyte glycoprotein (MOG) can cause a spectrum of autoimmune disorders, including optic neuritis, transverse myelitis, brainstem encephalitis, and acute disseminated encephalomyelitis. In this study, we present the case of a 19-year-old woman with an unusual clinical presentation of intracranial hypertension (IH) and bilateral papilledema. The patient presented with symptoms of increased intracranial pressure, which followed a relapsing, remitting course over several months. Serial CSF studies showed an increased opening pressure during clinical relapses. The CSF and serum tested positive for MOG immunoglobulin G antibodies. Contrast-enhanced MRI of the brain showed mild meningeal enhancement in the left parietal region with subtle underlying cortical hyperintensities, indicating possible fluid-attenuated inversion recovery variable unilateral enhancement of the leptomeninges. The patient responded well to immunosuppressive therapy using rituximab. The presentation of MOG antibody-associated disease (MOGAD) as IH without optic neuritis is rare. This report presents the first description of a relapsing remitting course presenting each time with only symptoms of raised intracranial pressure, without developing any typical clinical manifestations of MOGAD.