Purpose: Craniosynostosis is defined as the premature fusion of any of the cranial sutures. It may occur sporadically or with other anomalies in a syndrome. Synostosis of bilateral squamosal sutures is a rare condition that has only been reported in a handful of cases in current literature. Loey-Dietz syndrome is a rare condition with vascular, skeletal, and cutaneous manifestations overlapping with the more commonly known Marfan’s syndrome. Methodology: We report the first known published case of bi-squamosal craniosynostosis in a patient with Loey-Dietz syndrome. Results: An 18-month-old female was referred to a craniofacial unit with scaphocephaly. She was born at 36 weeks with a maternal history of Loey-Dietz syndrome. Genetic testing revealed SMAD3 mutations consistent with a genetic diagnosis of Loey-Dietz syndrome. CT imaging diagnosed bilateral squamosal suture synostosis. Fundoscopy demonstrated no papilloedema. The patient was managed with multidisciplinary input with neurosurgery, ophthalmology, and cardiology specialties. No surgical intervention has been required for her craniosynostosis at this stage and she continues to be managed through a multidisciplinary unit. Conclusion: This is the first known case of syndromic bi-squamosal suture synostosis in a patient with Loey-Dietz syndrome. Loey-Dietz syndrome is an autosomal dominant syndromic condition diagnosed through a combination of phenotypic clinical features as well as a genetic mutation in genes affecting the transforming growth factor-beta pathway (TGFBR, TFB and SMAD mutations). This case highlights the need to recognise this as a rare cause of syndrome craniofacial anomalies and the need to manage this in a specialised multidisciplinary setting.