Osteogenesis Imperfecta (OI), also known as “brittle bone disease”, is a rare autosomal dominant genetic disorder (with 4 subtypes) affecting connective tissue and is characterized by extremely fragile bones that fracture easily often without apparent cause. The genetic mutation has been identified as affecting 1 of 2 genes, COL1A1 or COL1A2, located on chromosomes 17 and 7 respectively. These genes are involved with the production of type I collagen. Collagen is a major protein of bone and connective tissue. In OI, there is a 10-30% incidence of a bleeding diathesis in patients undergoing surgical procedures [1,2]. Нe coagulation abnormality appears to be associated with the abnormal collagen adversely aوٴectLnJ platelet-endothelial cell interactions and capillary strength. Friable soÑ– tissue with small blood vessels unable to vasoconstrict adequately and a defect in the platelet aggregation response around exposed subendothelium are felt to be a result of the collagen defect in OI. Нese patients can have increased capillary fragility, decreased platelet retention, decreased levels of factor VIII and deficLent collageninduced platelet aggregation that can be LdentLfied through laboratory studies [3]. Unfortunately and even more concerning excessive bleeding can occur despite normal coagulation and platelet function studies.