This case provides clinicopathological evidence supporting the association between FBDS and basal ganglia involvement in anti-LGI1 encephalitis. It highlights the diagnostic challenge posed by tumour-like unilateral basal ganglia lesions on MRI and underscores the importance of neuronal autoantibody testing before invasive procedures. In addition, this report contributes the first neuropathological description of basal ganglia involvement in anti-LGI1 encephalitis and summarises previously reported pathological findings.