For routine clinical practice, it is vital to plan the steps same as possible complications that may occur and how to solve them before surgery. This case is of special interest for being a low-incidence pathology and a rarely used repair for the defect. The case is of high interest because of the large size of the defect and the complexity of managing and repairing the skull shell. This case highlights the importance of considering different possibilities, even two-step surgery when it comes to large defects on the skull after craniosynostosis diagnosis.