This study aimed to comprehensively describe the initial presentations and subsequent clinical courses, ancillary findings, treatments, and outcomes of patients with anti-KLHL11 encephalitis.<h4>Methods</h4>We tested 1,361 patients with clinical features or tumors that could be associated with anti-KLHL11 encephalitis (1,164 CSF and 680 serum). Here, KLHL11 is linked to encephalitis.