CASE REPORT: A 3 years-old boy, brought by his mother who noticed a mass in the external auditory canal of the left ear. Otoscopy: presence of otorrhea and a red, polypoid lesion obliterating the external auditory canal. The mass was painless and its insertion was sessile. Imaging study (CT scan and MRI) was carried out and revealed an extensive erosion of the bone of the external auditory canal and an extention to the infratemporal fossa. Biopsy was done trought the endoaural approach. The anatomopathological examination revealed lymphohistiocytic infiltrate without atypia and immunohistochemical analysis was compatible with Kimura's disease. CONCLUSION: Althoug rare, Kimura's disease should be part of the differential diagnosis of temporal bone lesions.