Flavonoids exist as aglycones, glycosides, or dimeric forms called biflavonoids. Biflavonoids presence is commonly reported in plants used in traditional and modern medicine, indicating their potential health benefits. Biflavonoids are not present in all plant species, but are found only in some species. One of them is ginkgo (Ginkgo biloba L.), a well-known medicinal plant used in traditional and Western medicine. Unfortunately, most phytochemical studies published on ginkgo in recent years have neglected biflavonoids as important bioactive components that have come into scientific focus as important health-promoting compounds. So, the aim of this work was to conduct a comprehensive literature review on the presence of biflavonoids in ginkgo and to summarize data on the chemistry, diversity, and biological factors affecting their presence in ginkgo, as well as their bioactive and health-related properties. To date, the presence of 13 biflavonoids in ginkgo has been demonstrated, of which amentoflavone, bilobetin, sciadopitysin, ginkgetin, and isoginkgetin are the most common. Their role in plants remains unknown, but their bioactivity and potential role in treating human health are better understood and are associated with antioxidant, anticancer, antiviral, antibacterial, antifungal, and anti-inflammatory activities, as well as their potential role in treating cardiovascular, metabolic, and neurodegenerative diseases.