Craniosynostosis is a common craniofacial anomaly, with an estimated incidence of 1/2000 to 1/3000 births. Scaphocephaly accounts for 40-58% of all non-syndromic single-suture synostosis. Only 2-9% of these are familial cases. We report the clinical findings in a three-generation family presenting with familial scaphocephaly. Case presentation: The patient was born at 41 weeks gestation by vaginal delivery. A ridged sagittal suture was discovered on clinical exam at three months of life. A CT scan of the skull with 3-D reconstructions confirmed the diagnosis. He underwent cranial vault remodeling. His 28 year-old father was also diagnosed with sagittal suture synostosis at the age of 5 months and underwent urgent craniotomy for increased intracranial pressure. His paternal grandfather as well as his paternal great uncle and a first cousin to the father all have the diagnosis of sagittal suture synostosis. None of them have had surgery. We describe the clinical findings and family tree of a familial case of sagittal craniosynostosis. A mutation in the fibroblast growth factor receptor 2 gene (FGFR2) tyrosine kinase domain (versus other sites) could be the cause for this familial presentation. The related clinical and genetic literature will be reviewed.