Many ABC proteins are functional in cellular lipid transport in various different manners. ABCA7 is a full-size ABC transporter, the physiological function of which is unknown to date. This is a protein that shows the highest homology known to ABCA1, an essential molecule for producing of plasma high-density lipoprotein (HDL), and in fact it mimics ABCA1 to mediate the production of HDL from cellular lipid when transfected in vitro. It is therefore rational to assume that ABCA7 plays a relevant role in regulating of lipid metabolism. However, the ABCA7 expression profile is distinct from that of ABCA1, with respect to tissue-specific distribution and response to some reagents, presumably because of different transcriptional and posttranscriptional regulation. Potential roles and functions of ABCA7 in lipid homeostasis are discussed, especially in relation to HDL metabolism, based on available publications.