Osteogenesis imperfecta (OI) constitutes, within the group of bone dysplasias, a set of congenital disorders of collagen type 1 fibers. These mutations generate a biochemical defect that translates into a complex clinical picture: low stature, bone deformities, osteopenia, respiratory and cardiovascular problems, and dentinogenesis imperfecta. The 3 main lines of treatment for OI within which nursing care is developed are: a) to prevent and control the clinical manifestations of the disease, so that normal development of the child is encouraged; b) to promote maximal physical, emotional and social independence of sufferers, preventing the development of complications such as fractures and deformities, and c) to provide health education to sufferers and those in their environment, especially their parents. The aim of the present article is to provide, through a case study, basic knowledge this disease, which is of great therapeutic importance because of its severe associated deformities and disabilities.