Osteopetrosis is a rare congenital bone disorder, characterized by systemic osteosclerosis due to a deficiency of or functional defect in osteoclasts. Autosomal dominant osteopetrosis (ADOP) is the most common form with a late onset, a stable condition, relatively few symptoms and a good prognosis. Few studies to date have reported successful total hip arthroplasty (THA) in patients with ADOP and its operative difficulties. We herein describe a case of left hip osteoarthritis in a patient with OP via THA in order to share our experience during the treatment process. The patient was a 52-years-old female with osteopetrosis who was referred to our department due to a history of left hip pain with activity limitation for 20 years. The patient reported no history of fracture or family history. The patient underwent THA in the left hip. At the 6-month, 1-and 2-year follow-up, the components were in a good position and the patient remained asymptomatic and pain-free. Therefore, THA may be a feasible treatment option when patients with ADOP suffer from painful hip osteoarthritis.