Introduction: Solitary median maxillary central incisor syndrome is rare, with only single reports in the literature. This congenital defect affects both primary and permanent teeth. It is rarely an isolated anomaly, but usually a set of general symptoms. Objectives: The aim of the study was to collect reports on single incisor syndrome and analyze them in the context of our own case. Materials and methods: We assessed available literature reports on solitary median maxillary central incisor syndrome, including case reports. We also presented our own case based on systemic, dental, and radiological examination. Results: The described case was a single maxillary incisor syndrome confirmed by dental and radiological examinations. The patient presented with oral symptoms typical of the disorder, and a systemic interview confirmed extraoral symptoms. Conclusions: Solitary median maxillary central incisor syndrome is a rare midline defect that is usually not an isolated genetic disorder. The multitude of symptoms and disorders throughout the body requires an interdisciplinary and individualized approach to the patient.