Abstract: Misfolded proteins accumulate in cells to form protein aggregates, which interfere with the normal physiological activities of cells and cause various human diseases, especially neurodegenerative diseases. Therefore, studying the process of aggregate formation and clearance is significant for the therapy of neurodegenerative diseases. Protein aggregates are regulated by phase separation during their formation, transitioning from liquid to solid states, which affects the properties and removal of aggregates. Although a variety of intracellular protein quality control systems can be involved in the removal of misfolded proteins, protein aggregates need to be cleared through the autophagy pathway, and the process is called aggrephagy. In aggrephagy, autophagy receptors such as p62, NBR1, TAX1BP1, Tollip, and CCT2 can help the autophagy system to recognize protein aggregates, thus plays an important role in the removal of aggregates. This article will introduce the formation and autophagic clearance of aggregates, with a focus on the latest research progress in the field of aggrephagy.