The clinico-pathologic, immunohistochemical and radiological features of 12 jaw cysts with a prominent orthokeratinized epithelial lining were studied and compared with those of typical odontogenic keratocysts and dentigerous cysts. They differed significantly from odontogenic keratocysts in terms of biologic behavior and histopathologic findings. Although immunohistochemical staining of the epithelial linings for cytokeratins, EMA, CEA and involucrin has not shed any light on the histogenesis of these lesions, staining patterns for these markers were significantly different from those of odontogenic keratocysts and non-keratinized dentigerous cysts. Radiologically, nine cases appeared as dentigerous cysts; two cases, one with sebaceous differentiation, as non-dentigerous unilocular cysts, and the remaining one was exceptional as it showed multiple epidermal cysts with prominent dermal appendages histologically. It is suggested that most of the orthokeratinized jaw cysts may belong to clinico-pathological entities different from odontogenic keratocysts with the majority representing dentigerous cysts with orthokeratinization. The possibility of the existence of rare central dermoid or epidermoid cysts is also to be considered.