Abstract: This article describes the first published case of coexistence in a child of a rare hybrid odontogenic ghost cell tumor and a solitary cutaneous pilomatrixoma. An 11-year-old boy presented with a large well-defined unilocular radiolucent lesion in the right posterior mandible. Marsupialization followed by enucleation of the remaining lesion at a later period was the treatment of choice. Histopathologic analysis revealed a hybrid tumor demonstrating areas identical to calcifying cystic odontogenic tumor, ameloblastoma, ameloblastic fibro-odontoma, ameloblastic fibromyxoma, and adenoid odontogenic tumor. A cutaneous nodule was also removed from the facial area and demonstrated classic features of pilomatrixoma on histopathology. Sixteen cases of hybrid calcifying cystic odontogenic tumor associated with odontogenic tumors other than ameloblastomas and odontomas are referred in the literature to date. Young males are frequently affected, and the mandible is the most common site of involvement. The occurrence in the same patient of 2 distinctive entities, which both demonstrate ghost/shadow cells, may be a coincidental finding or suggest a common origin regarding the histogenesis of these cells. Alternatively, future molecular studies may clarify possible genetic or/and predisposing factors for the development of these lesions.