Recognition and evaluation of vascular Ehlers-Danlos syndrome (vEDS), osteogeneisis imperfecta (OI) and the recently described vEDS-OI crossover syndrome is critical in safe surveillance and treatment of these patients. We present an individual with type B aortic dissection, carotid dissection and a COL1A1 gene mutation in a family with phenotypic features of osteogenesis imperfecta. Aortopathy in patients with osteogenesis imperfecta is exceedingly rare and concomitant cerebrovascular disease has not previously been described. Members of this family exhibit variable but distinct phenotypic features highlighting the importance of genetic testing and multi-disciplinary management by the vascular surgeon as the contemporary literature in this area continues to evolve.