Pleiomorphic adenoma genelike 2 (PLAGL2), together with pleiomorphic adenoma gene 1 (PLAG1) and pleiomorphic adenoma genelike 1 (PLAGL1), belong to the zinc finger protein gene family. They are highly homologous in structure, and the differences in function are mainly due to different DNAs they bind. In recent years, great progress has been made in the research of structure, function and mechanism of PLAG1, PLAGL1 and PLAGL2. PLAGL2 has been found to be related to cell metabolism and development of many systemic diseases, while there exists controversy on the role of PLAGL2 in tumors. The research progress of structure, transcription mechanism and biologic function of PLAGL2 and its relationship with systemic diseases and tumor are reviewed in this paper.