A 25-year-old woman, previously healthy, in her 17th week of pregnancy, presented to a local hospital with acute onset of memory impairment, right upper extremity clumsiness, task apraxia and right-sided neglect. Patient was transferred to a tertiary referral center after non-contrasted brain MRI showed restricted diffusion with FLAIR abnormality in the subcortical left parieto-occipital area concerning for atypical RPLS versus demyelinating lesion versus malignancy. Lumbar puncture (LP) was performed without obvious infectious or inflammatory results. Examination was notable for severe right-sided neglect, right homonymous hemianopia and Gerstmann’s syndromic features. Infectious, metabolic, and rheumatologic panels were negative. Over 24 hours, patient worsened with severe bilateral neglect and bilateral central sparing homonymous hemianopia. Repeat non-contrasted brain MRI, 5 days from the initial, revealed bilateral parieto-occipital involvement with extension across the splenium of corpus collosum to the right. Repeat LP showed mild lymphocytic pleocytosis, normal protein, positive oligoclonal bands and an elevated IGG index. She was treated with high dose steroids, plasmapheresis and rituximab with clinical improvement and a repeat brain MRI showing stable lesions without radiological progression.