Craniosynostosis isolated to the frontosphenoidal suture is an extremely rare entity, distinct from other types of synostotic anterior plagiocephaly, from the embryologic, phenotypic and endocranial morphology viewpoints. Embryologically, the sphenoid bone has two origins, which morphogenetically represent 2 distinct subunits. Depending on the region involved, unique craniofacial features involving the forehead, temporal region and orbit will be demonstrated. A case of frontosphenoidal suture synostosis depicting these features is presented. In addition, a literature review was performed and a treatment algorithm is proposed.