Mountain regions supply a large amount of fresh water for the people in arid and semiarid regions, however, there is great uncertainty of the water quantification from mountains to lower areas. In order to assess the hydrological significance of mountains and the hydrological linkage of mountains and plains, the measured and simulated hydrological data of the arid region in northwest China were used in the present research which followed a catchment-based approach. Firstly, the Heihe River Basin, a welldocumented area, was selected as a specific watershed to reveal the hydrological relationship between highlands (mountains) and lowlands (plains); and then, the significance and disproportion of mountain runoff of 8 river basins as cases in the arid region of northwest China were analyzed and compared following the above analysis. The results of the study showed that the proportion of mountain runoff in total basinal runoff (PMR) of most rivers is above 50%. The PMR are between 50%-95% in the rivers originated in the northern slope of the Tianshan Mountains where the aqueous vapor is relatively sufficient. And that, almost all the flow of the rivers originating from the Qilian Mountains, the southern slope of the Tianshan Mountains, and the northern slope of the Kunlun Mountains come from mountain regions. Also the PMR gradually increases from west to east in northwest China. The hydrological significance and disproportion of mountains water in the arid region of northwest China were given a systematic and thorough assessment, and the results could give potential guides for the scientific utilization of water resources in these regional areas for relieving the more and more serious shortage of water resources due to climate warming and population expansion. Mountain, an important water-source region, can effectively supply water resources to its neighboring lower hills and plains. Not only abundant rainfall, there are also a large number of glaciers and snow storage in the high-altitude mountains. The melting water from glacier makes up large amount of water resources. Most rivers on the Earth have their sources in mountain regions. The relationship between mountains and plains in water balance is an important scientific problem. There were many researches about that and had a lot of results, especially in the glaciology and climatology [1][2][3][4][5][6][7][8]. Though the existing researches have actively promoted the development of mountain hydrology and the studies in water balance linkage of mountains and plains, there are still many contradictions and problems needed to be solved, such as the shortage of the systematic researches and quantificational describes about hydrological significance of mountains. The arid region of northwest China, located in the hinterland of the Eurasia, covers approximately 24.5% of China's total land area, and about 43.5% of it are mountains and hills [9][10][11]. Little precipitation with vast evaporation makes there become one of the world's most severe drought