Solitary median maxillary central incisor (SMMCI) syndrome is considered as a microform of holoprosencephaly (HPE) spectrum. It is a unique developmental abnormality affecting midline structures of the body. Systemic abnormalities involve major systems of the body such as cardiovascular, gastrointestinal, renal, endocrine, genital, and skeletal systems. Abnormal features involving eyes, ears, nose, mouth, and skin are also present. SMMCI patients belong to the HPE spectrum, and hence are at risk of having children with a more severe form of HPE. Very often SMMCI patients may first be seen by a pedodontist or an orthodontist and it is important that the dentist performs a complete examination to rule out systemic abnormalities and involve a multidisciplinary team to manage the patient. This article reports about a 5-yearold male child who reported with dental abscess in whom SMMCI was an incidental finding. Further investigations revealed the association of SMMCI with anomalies such as chronic respiratory obstruction, short stature, abnormal clinical and radiographic craniofacial morphology, duplicated left thumb phalynx, and absent left testis.