Desmoplastic fibroma (DF), a rare benign locally aggressive neoplasm of bone composed of bland spindle cells and abundant collagen. In this report, we present a case of DF in the mandible. Differential diagnosis and treatment strategy are discussed with a literature review. An 11-year-old male patient with swelling and pain complaint in left mandible visited our clinic. As a result of radiological and clinical examination, a well and sclerotic demarcated lytic lesion was detected.A computed tomographic scan demonstrated 3 × 11cm multifocal expansive lesions at the left mandible. The lesion has excised. As a result of histopathological examination the lesion turned out to be DF. Although we know that recurrence rates raises to 55-72% from 17% when local excision preferred over hemimandiblectomy or segmental resection; to do not disturb the natural maxillofacial skeletal progress. A periodic follow-up is warranted.