There has been considerable controversy about the relation between Kimura's disease and angiolymphoid hyperplasia with eosinophilia (ALHE). We describe the clinical and histopathologic differences between the two diseases. We reviewed clinical findings and histopathologic changes in two cases of Kimura's disease and three cases of ALHE. Kimura's disease shows typical lymphoid follicles. It is associated with lymphadenopathy and is always accompanied by peripheral eosinophilia. ALHE shows typical changes in endothelial cells. It is characterized by superficial papules or nodules with no lymphadenopathy and is less frequently accompanied by peripheral eosinophilia. Kimura's disease and ALHE are separate entities.