Introduction: Syndromic craniosynostosis with growth restriction often causes increased intracranial pressure requiring shunting. Crouzon syndrome also known as branchial arch syndrome is an autosomal dominant disorder which has multiple phenotypic characteristics including proptosis, low set ears, bilateral strabismus, orofacial deformities affecting maxilla and mandible, and most importantly abnormal fusion of skull sutures also known as craniosynostosis.
Case Report: A 17-year-old male presented with severe Crouzon syndrome and brachycephaly who had a delayed cranial vault expansion. He subsequently developed intermittent shunt malfunction, which progressed to a complete obstruction. After successful endoscopic third ventriculostomy and return to baseline function the shunt was tied off.
Conclusion: Endoscopic third ventriculostomy (ETV) can be performed safely and effectively for shunt malfunction after cranial vault expansion in patients who may not have been candidates previously. By using ETV in addition to known traditional techniques of cranial vault expansion, patient’s prognosis and postoperative recovery can be expedited.

Keywords: Craniosynostosis, Crouzon syndrome, Expansion, Vault