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Objective: Oral and maxillofacial lesions vary regarding their clinical presentation in different populations. Until now, oral and maxillofacial lesions in Iranian children and adolescents have not been studied. The aim of this study was to determine the type and distribution of biopsied oral lesions among children and adolescents in Southern Iran. Methods: All the patients referred to the pathology department of Shiraz Faculty of Dentistry from 1991-2009 were enrolled in this retrospective, case-series study. The information regarding the patients ’ age, gender as well as the histopathologic type and anatomic location of the biopsied oral lesions in patients under 18 years was collected from patients ’ medical documents and were analyzed by SPSS version 11. Findings: Out of 2984 patients, 576 (19.3 %) cases were children and adolescents under 18 years. The most prevalent category was soft tissue lesions (45.5 %). The most common lesion was peripheral giant cell granuloma (15.6%) followed by dentigerous cyst (14.2%) and pyogenic granuloma (11.3%). Gingiva was the most common affected site. Male to female ratio was 1.2. Conclusion: Our results revealed that near 20 % of orofacial lesions occur in children and adolescents with rather equal male to female ratio. The majority of lesions were soft tissue diseases with a reactive nature