Diagnosis of odontogenic tumors can be challenging due to their rarity and diverse morphology, but when arising near the tooth, the diagnosis could be suspected. When their location is not typical, like inside the paranasal sinuses, the diagnosis is less easy. Maxillary ameloblastomas are exceedingly rare with only sparse information on their epidemiological, histological and genetic characteristics. The aim of this report is to thoroughly review the available literature in order to present the characteristics of this tumor. According to available data, maxillary ameloblastomas can occur in all ages but later than mandible ones, and everywhere within the maxillary region without necessarily having direct contact with the teeth. No sex preference has been shown. The most common histological patterns seen in this location are the follicular and plexiform ones. Maxillary ameloblastomas are locally aggressive neoplasms, thus therapy aims for excision including normal bone beyond the lesion. In contrast to mandible ameloblastomas, maxillary ones most commonly show mutations of the SMO gene. Furthermore, differential tumor diagnosis is thoroughly discussed in the present review. Odontogenic tumors are rare and can pose diagnostic difficulties for non-specialized pathologists. Nevertheless, when the location is typical, i.e. molar region and when the histology shows the characteristic features of odontogenic neoplasms, diagnosis could be straightforward. In locations outside the oral cavity or when rare histological variants are found, suspecting the correct diagnosis can be challenging. This is especially true for maxillary ameloblastomas, which are rare, possibly leading to low awareness of this neoplasm at this location and often show non-classical morphology, thus, rendering its diagnosis more complicated. Thus, the aim of this review is to define and thoroughly describe maxillary ameloblastomas based on the available literature after a short introduction in the entity of ameloblastoma.