Abstract Background A review of the literature revealed that only 9 total knee replacements were performed on patients with osteogenesis imperfecta (OI), with one being a revision procedure of a periprosthetic fracture. Of the 9 primary procedures, all used cemented prostheses, and 3 patients had an osteotomy at the same procedure. Our patient required a hinged prosthesis because of collateral ligament incompetence and is the first such case reported in the literature. Case presentation Presented here is a total knee replacement performed on a 52-year-old patient with osteogenesis imperfecta (OI) who injured her left knee and ruptured her anterior cruciate ligament. Her right knee suffered from severe degenerative changes with an incompetent medial collateral ligament. It was decided to replace the right knee before addressing the left knee injury. A hinged revision prosthesis was used. The smallest components available were used because of the small anatomical bony dimensions. Conclusion This is the first reported case of a hinged prosthesis and highlights the soft tissue component of osteogenesis imperfecta. We also highlight the technical problems with these patients, including mal-alignment, small bony dimensions and bone fragility.