We herein report an autopsy case of relapsing polychondritis encephalitis coexisting with a Lewy body pathology and also review previous autopsy cases. A 59-year-old man exhibited a tremor of the right hand, small-steppage gait and bradykinesia. Five years later, he presented with relapsing auricular chondritis and scleritis and subsequently showed exacerbation of extrapyramidal symptoms. A histological examination revealed perivascular lymphocytic cuffing and infiltration in the small vessels, as well as loss of nerve cells and gliosis in the basal ganglia, insular gyrus and medial temporal lobe. The present case was characterized by perivasculitic panencephalitis and the coexistence of a Lewy body pathology, which may have augmented the patient's parkinsonism.