To evaluate the cognitive and neurofunctional outcomes in patients with anti-leucine-rich glioma inactivated 1 (LGI1) encephalitis.A cohort follow-up study was performed after a median of 33 months (range 6-78) from disease onset to the last follow-up in patients diagnosed with anti-LGI1 encephalitis, to assess the neurofunctional outcomes using modified Rankin Scale (mRS), activities of daily living (ADL), neuropsychiatric inventory (NPI) and modified telephone interview for cognitive status (TICS-M). Remote symptomatic seizure and clinical relapses were also recorded. The clinical, laboratory features, and treatment responses that characterize the disability were analyzed.The results showed that 81 of 86 (94.2%) patients with anti-LGI1 encephalitis were successfully followed up, while eight (9.9%) died after discharge. Among the 73 survivors, clinical relapses occurred in 18 (24.7%) patients, and those with relapses were at a higher risk of developing remote symptomatic seizure (p = .019). Although 85.2% of the patients became functionally independent (mRS ≤2), the sequelae of symptomatic seizure, neuropsychiatric symptoms, and cognitive deficits were found in 11.0%, 21.9%, and 39.7% of the patients, respectively. Residual cognitive deficits primarily occurred in the elderly subjects as well as those with symptoms of memory deficit, psychiatric disorders, sleep disturbance, disturbance of consciousness at diagnosis, and higher CSF protein levels.Although most patients survived and became functionally independent, a subset of patients could not return to all premorbid activities. They may have clinical relapses or suffer from remote symptomatic seizure, neuropsychiatric symptoms, and cognitive impairment.© 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.