Due to an increasing lifespan, patients with osteogenesis imperfecta have a high incidence of hip osteoarthritis. The presence of recurrent fractures and deformities make primary and particularly revision total hip arthroplasty challenging. We present a series of patients with osteogenesis imperfecta undergoing total hip arthroplasty at a tertiary referral centre with a median follow-up of 7.6 years (4 to 35 years). There were four primary total hip arthroplasties and eight revision total hip arthroplasties performed in four patients. Three femoral components were custom computer assisted design computer assisted manufactured. The survival rate of the primary total hip arthroplasty was 16% and there were ten complications: five intraoperative fractures, one case of septic loosening and four cases of aseptic loosening. Patients with pre-operative acetabular protrusio were significantly more likely to require revision surgery (p = 0.02). At latest follow-up, the median Oxford hip score was 41 (37 to 46). As the largest series of primary and revision total hip arthroplasty performed in patients with osteogenesis imperfecta, we report good medium to long-term outcomes. Preoperative planning and consideration of custom made prostheses have an important role in these complex cases.