Annexin A3 (ANXA3), an annexin family member, contains 36 kDa and 33 kDa isoforms. Similar to other annexin members, ANXA3 plays an important role in the development of human diseases. Recent studies have reported that abnormal ANXA3 expression is closely associated with the development, progression, metastasis, drug resistance and prognosis of several malignant tumours, such as breast cancer, lung cancer and hepatocellular carcinoma. ANXA3 exerts its role by regulating cell proliferation, migration and apoptosis via the phosphatidylinositol-3 kinase/Akt, nuclear factor-κB (NF-κB), c-JUN N-terminal kinase, extracellular signal-regulated kinase and hypoxia-inducible factor-1 signalling pathways. ANXA3 may act as a novel target for the early diagnosis and treatment of tumours. The present review summarises the recent progress in the role of ANXA3 and its regulatory pathways in tumours.