【Objective】 To investigate the distribution of ABO blood type of patients in Naqu, northern Tibet Plateau. 【Methods】 Blood group data of 5258 patients who underwent blood group identification in People′s Hospital of Naqu from 2017 to 2019 were retrospectively collected. Statistical analysis and Hardy-Weinberg coincidence test were conducted, and the disease profile, including anemia, high altitude polycythemia, obstetrics & gynecology, etc., of patients with different blood types was analyzed. 【Results】 Analysis showed that type O was most prevalent with the proportion of 37.73%(1 983/5 258), following with type B 34.12%(1 794/5 258), type A 21.3% (1 120/5 258)and type AB 6.85%(360/5 258). The distribution of ABO blood group was in accordance with Hardy-Weinberg equilibrium law, showing a trend of O>B>A>AB, and the trend of gene frequency was r>q>p. The distribution of ABO blood group in male and female patients was basically the same, and showed the trend of O>B>A>AB. The distribution of ABO blood group in Naqu area was similar to other high altitude areas of Tibet (P>0.05), but presented a certain difference with that in low altitude areas of Tibet (P<0.05). The distribution characteristics of ABO blood group in anemia, high altitude polycythemia and obstetrics & gynecology patients were also in accordance with Hardy-Weinberg equilibrium, showing a trend of O>B>A>AB. Blood group distribution of patients with different systemic diseases was different, but with no statistical significance. 【Conclusion】 The profile of ABO blood group of patients in Naqu area is consistent with that in high altitude areas of Tibet, but quite different from that in low altitude areas of Tibet. Knowing the distribution characteristics of ABO blood group of patients in this area (hospital) is helpful to formulate a set of safe and reasonable blood use (storage) plan and ensure clinical blood use, especially emergency blood use.