Plagiocephaly is a common condition that affects infants. It can be broadly grouped into positional and non positional plagiocephaly Positional plagiocephaly frequently resolves without intervention. Non positional plagiocephaly resulting from craniosynostosis often requires surgical intervention. In this case report, we present a rare case of unilateral frontosphenoid craniosynostosis. We discuss the appropriate diagnostic workup, the available treatment options, and patient follow-up over time. Furthermore, we provide a detailed review of the literature discussing treatment options for aesthetic appearance as the child ages.