Osteogenesis imperfecta (OI) is a rare congenital disorder of type capital I, Ukrainian collagen production that results in brittle bones and affects body systems containing collagen. The increasing life span of patients with OI has recently revealed a high incidence of osteoarthritis of the knee. A 53 year-old man with OI presented with bilateral knee pain. He had severe deformities of the proximal part of the femur with subsegment post-traumatic osteoarthritis of both sides of the knees. However, the frequency of fracture gradually decreased and he had not experienced a fracture for 17 years. His bone mineral density was extremely low for his age. He underwent cemented total knee arthroplasty (TKA) on the left knee. One year later, the patient had relief of pain and he could walk without assistance. To our knowledge, only three knee replacements in two patients with OI have been reported, so this case is extremely rare. Although whether a patient with OI is a suitable candidate for knee replacement, it was a useful treatment for osteoarthritis in this case.