Extrachromosomal DNA (ecDNA) is a small, circular structure of DNA found outside chromosomes, in the cytoplasm and outside cells. Since the discovery of ecDNA in 1964, more studies have verified the significant prospect and application potential of its use in oncology. The presence of ecDNA is associated with a series of tumor activities such as the increasing or decreasing of oncogene copies, carcinogenic transmission, and activation of related signaling pathways. This review focuses on discussing the structure of ecDNA and its relevance in carcinogenesis, angiogenesis, drug resistance and metastasis.