Isolated frontosphenoidal craniosynostosis is extremely rare, due to which diagnosis can be challenging. All the isolated cases reported have been treated by open surgical technique. We present a unique case report of an infant with an isolated frontosphenoidal synostosis corrected by a novel endoscopic surgical technique. The patient was a 5-month-old boy with worsening abnormal head shape indicating progressive right frontal bossing, left orbital displacement, and right occipital plagiocephaly. Computed tomography scan confirmed the diagnosis of left isolated frontosphenoidal craniosynostosis. Endoscopic-assisted surgical release was done starting with a 2.5-cm incision in the left temple area followed by a 2-cm strip osteotomy to excise the fused frontosphenoidal suture. Custom-made orthotic helmet therapy was started 2-weeks postsurgery, with excellent progress noted in the head shape confirmed by laser scans. The benefits of the endoscopic technique include smaller incision, decreased blood loss and need for blood transfusion, and decreased stay in hospital. This is the first case report showing the application of a novel endoscopic-assisted surgical treatment in an isolated frontosphenoidal craniosynostosis with no complications noted.