Acyl-coenzyme A binding domain containing 3 (ACBD3) is a scaffold protein of the Golgi apparatus that participates in various protein-protein interactions. ACBD3 plays a wide range of biological roles in cellular function and signal transduction. It is involved in mitochondrial cholesterol transport and steroid synthesis, regulating processes such as pathogen replication, cell apoptosis, and neurogenesis. Furthermore, ACBD3 plays crucial roles in the occurrence and development of diseases, such as tumors and Huntington' s disease. In this article, we review the research progress on the structural characteristics and physiological functions of ACBD3.