According to the 2005 histological classification of odontogenic neoplasms by the World Health Organization, ameloblastoma is a benign, locally invasive epithelial odontogenic tumor of putative enamel organ origin. There are four distinct subgroups in which this neoplasm can be gathered: the solid/multicystic type, the unicystic type, the desmoplastic and the peripheral type. Peripheral ameloblastoma is believed to be the rarest subgroup, making up for 2 to 10% of all ameloblastomas. From its first description by Kuru in 1911 to date, less than 200 cases of PA have been described in literature. PAs commonly affect the mandible, in the maxilla the most common location is the soft palatal tissue of the tuberosity area. The present report discusses a rare case of PA aroused in the gingiva of upper jaw in a 64-year-old woman. The treatment of the lesion and its immunohistochemical phenotype are described. A review of the literature is also performed, focusing on the epidemiological and pathological aspects of the lesions and their implications on the therapy.