Korla fragrant pears are one of the “famous, excellent, and special” fruits in Xinjiang. They belong to the white pear variety in the genus Pyrus of the Rosaceae family. With a long-standing planting history and strong regional characteristics, they are mainly produced in southern Xinjiang. Due to unique natural conditions such as large temperature differences between day and night and sufficient sunlight, Korla fragrant pears have a crispy texture, a sweet but not cloying taste, and their flesh is delicate and juicy, with excellent quality. Korla fragrant pears contain a variety of bioactive substances, mainly including polysaccharides, polyphenolic compounds, flavonoid compounds, triterpenoids, and sterols. They have medical effects such as “moistening the lungs, calming the heart, reducing phlegm, anti-inflammation, relieving cough, and resolving carbuncle toxins”. Uyghur and Mongolian medicine often use them as a good dietary therapy product. Based on recent literature reports, this paper reviews the main chemical constituents and pharmacological effects of Korla fragrant pears, aiming to provide references for the research and utilization of the deep processing of Korla fragrant pears.