Osteoma is a rare, benign, bone forming tumour which can arise from various sites in the cranial vault including temporal bone. It is a slow growing tumour. In external auditory canal, normally it is asymptomatic although it may cause cosmetic deformities. Large size osteoma of external auditory canal causes obstruction of external auditory canal along with pressure symptoms such as headache. It must be distinguished from exostosis in external auditory canal, a much common condition in that site, since there is ample clinical and histopathological evidence that these two lesions are separate entities. The purpose of the present study is to present a review of literature about external auditory canal osteoma and to report in details the case of a 10 years old female patient with the lesion and focusing on differentiating features between osteoma of external auditory canal and exostosis.