The ameloblastoma is a true neoplasm of odontogenic epithelium which has been described as persistent, locally invasive tumor having aggressive but benign growth characteristics. Several clinico-radiological and histological types are included in the ameloblastoma. Apart from the most commonly encountered clinico - pathologic models there are few variants, whose biological profile is unknown or not elicited. Unicystic ameloblastoma (UCA) is one of the types which refers to those cystic lesions that show clinical, radiographic, or gross features of a mandibular cyst, but on histological examination show a typical ameloblastous epithelium lining part of the cystic cavity, with or without luminal and/or mural tumor growth. Here we report a case of UA in a 40-year-old male with detailed clinical, radiographical and histopathological features with differential diagnosis and treatment.