Odontogenic lesions have been described in several syndromes. Despite multiple reports of ameloblastoma's association with various syndromes, it has not been added to the diagnostic criteria for any syndrome. Thus, the aim of this systematic review was gathering such cases’ data. An electronic search in PubMed Central's database was performed. The search strategy was limited to human studies (case reports and case series), full-text English articles published from first of January 1975 until the end of 2018. Irrelevant articles or articles with inadequate information were omitted. Associated syndrome, patients’ age and sex, lesions’ location, radiographic features, and treatment modality were collected and analyzed. Ameloblastoma was reported in association with Gorlin syndrome (6 cases), epidermal nevus syndrome (2 cases), Gardner syndrome (2 cases), Simpson-Golabi-Behmel syndrome (1 case), and Williams syndrome (1 case). The commonest associated syndrome was Gorlin syndrome (50%) with maxillary and female predilections. Both cases of epidermal nevus syndrome (ENS) were female with mandibular involvement. In syndromes that odontogenic lesions are one of the diagnostic criteria, it is important to rule out ameloblastic changes. Although the relationship between ameloblastoma and these syndromes is not well known, any related information may be helpful in understanding the pathogenesis and the nature of this neoplasm.