Dolichoodontoid is defined as hypertrophy of the apical portion of the odontoid process of the axis. We report the first case of a dolichoodontoid process in a pediatric patient with other congenital anomalies. This rare entity has had three previous citations in the literature. Our patient presented with scoliosis in which subsequent MRI demonstrated a Chiari I malformation, a small thoracic syrinx and a dolichoodontoid process. The diagnosis of this malformation should be entertained in cases of craniocervical anomalies.