A solitary median maxillary central incisor (SMMCI) is rare and affected individuals may carry a potentially serious condition known as SMMCI syndrome. However, many of these cases do not receive proper attention because they are misdiagnosed as agenesis of the maxillary central incisor. The purpose of this manuscript is to report two cases of children with only one maxillary central incisor and draw diagnostic differences between the entities. A correct diagnosis is very important because if an SMMCI is confirmed, the patient should be referred for genetic counseling.