A new calvarial hyperostotic syndrome (CHS) in young bullmastiffs is described. Calvarial hyperostotic syndrome clinically resembles canine craniomandibular osteopathy (CMO) and human infantile cortical hyperostosis (ICH), but it is unique in that there is progressive and often asymmetric skull bone involvement, and the population affected appears to be only young, male bullmastiff dogs. Characteristic radiographic findings consist of cortical thickening of the calvaria with irregular, bony proliferation over the frontal, temporal, and occipital bones. Histopathological examination shows that the trabeculae of the calvarial diploë are thickened and contiguous with a sunburst-like pattern of subperiosteal trabeculae composed of woven and lamellar bone tissue, accompanied by loose fibrovascular tissue and a variable inflammatory response comprised predominantly of neutrophils. In 80% of the cases presented, the lesion was self-limiting. The etiology remains unknown; however, traumatic, neoplastic, and degenerative conditions do not appear to be primary factors in the etiopathogenesis of the syndrome. It may be that this syndrome has a familial component, similar to that described for CMO and ICH.