Background: Types A and B hemophilia are coagulation disorders associated with many complications. Osteoporosis is a skeletal condition characterized by the decreased density of normally mineralized bone. This study aims to determine the relationship between osteoporosis and hemophilic arthropathy in severe hemophilia patients over the age of 20 years in Kerman, Iran.Methods: We performed a cross-sectional study of bone density among 40 male patients with severe hemophilia. Lumbar spine and femoral bone mineral density (BMDs) were measured using a Dual Energy X-ray Absorptiometry (DEXA) scan. The T-scores for BMDs were computed and values from -2.5 to -1 were considered as osteopenia. Those less than -2.5 were considered to be osteoporotic.Results: About 42% of patients had normal BMD, 50% were osteopenic, and the rest were osteoporotic. The mean BMI, number of arthropathic joints, and the numbers of joint bleeding during the previous year were signicantly higher in osteoporotic patients than osteopenic and normal groups (P=0.05, =0.003 and=0.011, respectively). The mean for factor replacement, the number of joint bleeding episodes in the past year, and the number of arthropathic joints were signicant independent predictors of both spinal and femoral BMD. Their odds ratios (OR) were 1.29 (factor replacement), 1.17 (numbers of joint bleeding episodes), and 1.73 (number of arthropathic joints), which were signicant (P<0.05).Conclusion: Our results suggest that men with severe hemophilia have reduced BMD. Patients at risk are those with signs of hemophilic arthropathy. Because osteoporosis may complicate the future treatment of patients with hemophilia, screening of patients with hemophilic arthropathy for reduction of BMD and preventive therapies is highly recommended.