Background: Juvenile temporal arteritis has been proposed as an entity but remains controversial. Case Report: A 39-year-old male, who was otherwise asymptomatic, developed painless bilateral nodules in the temporal areas. His eosinophil blood count was 2,660/mm3 (31%), while the erythrocyte sedimentation rate was normal. Histologic examination of the lesion revealed non-giant-cell granulomatous inflammation with abundant eosinophil infiltration. The clinical and pathologic manifestations in our patient were different from those in classic temporal arteritis, which occurs almost exclusively in individuals over the age of 50 years, allergic granulomatosis and angiitis, and thromboangiitis obliterans. Eight cases of this disease have previously been reported in the literature. Conclusion: We consider that ‘juvenile temporal arteritis with eosinophilia’ is a distinct clinical and pathologic entity. The prognosis of the diseases is considered to be g