Abstract
Solitary median maxillary central incisor (SMMCI) syndrome is complex and usually develops 35-38 days postconception during the intrauterine period. A noteworthy discovery is that just one central incisor in the maxillary alveolus, found exactly on the centerline, is present in both deciduous and permanent dentitions with other congenital anomalies. Around one in every 50,000 live babies exhibits this abnormality. This report describes the case of a 13-year-old female patient with SMMCI syndrome with a complaint about an unsightly appearance due to a single large upper front tooth. We underline the importance of increasing clinician awareness of SMMCI syndrome and the need for a multidisciplinary approach to its care.