Ginkgo biloba seeds contain a variety of nutritional and functional components and have high edible and medicinal effects, making them one of the famous food and medicine homologous materials. But G. biloba seeds also contain some harmful ingredients, which have severely restricted the healthy development of the G. biloba seed food industry. Overconsumption of G. biloba seeds can lead to food poisoning. Ginkgotoxin (4′-O-methylpyridoxine, MPN) and ginkgotoxin-5′-glucoside (4′-O-methylpyridoxine-5′-glucoside, MPNG) are the main toxic ingredients of G. biloba seeds and have been studied intensively. This work tries to comprehensively summarize the research status of MPN and MPNG (collectively known as ginkgotoxins), building the scientific basis for further research on ginkgotoxins, with the aspiration to promote the healthy development of the G. biloba seed food industry. In this review, the isolation, identification, extraction, and detection of ginkgotoxins are first introduced. Then, the synthesis and distribution of ginkgotoxins are described and discussed. Finally, the methods used for removing ginkgotoxins and the effects of processing on the ginkgotoxins are analyzed and compared. Reducing ginkgotoxins while preserving the nutritional and functional components of G. biloba seed foods will undoubtedly be a pressing issue in further research.