Thirteen-year-old girl complaining of the swelling and pain on the left midface visited our dental hospital. On the radiographic examination, well-defined radiolucent lesion with hyperostotic border was found in the left maxilla accompanying with the external root resorption of the involved teeth and the displaced second molar. CT showed calcified bodies, thinning of hard palate, inferior orbital wall and lateral wall of nasal fossa, and thinning and perforation of the buccal plate of the maxilla. Enucleation and curettage of the lesion and nasoantrostomy was carried out and histopathologic examination mainly showed a solid tumor tissue composed of odontogenic epithelium and pulp tissues admixed with dentin and enamel formation. And some part of reduced follicular epithelium of tooth germ showed a change mimicking calcifying odontogenic cyst. Taken together, we concluded the lesion is an ameloblastic fibro-odontoma with a change of calcifying odontogenic cyst.