We report a longitudinal case study of a left-handed girl who underwent left hemispherotomy at 7 years for Rasmussen encephalitis (RE). Presurgical evaluation showed mild hemiparesis, no visual defect, and light neuropsychological impairment with short-term memory weakness. Language fMRI showed a right hemispheric dominance. Postoperatively, the patient exhibited right hemiplegia and hemianopsia but preserved intellectual capacities. She became seizure-free, and antiepileptic medication was discontinued. Long-term follow-up showed very high verbal intelligence at 11 years of age (VCI of 155) and improvement in working memory as well as language and reading abilities. Furthermore, a significant visuoverbal discrepancy became increasingly pronounced. Thus, early surgical treatment of epilepsy avoided the global cognitive deterioration usually associated with RE. Finally, such a high level of verbal functioning combined with low spatial reasoning with a single hemisphere provides additional information on the neurocognitive profile of children with RE after hemispherotomy.