The endoplasmic reticulum quality control (ERQC) system, a core mechanism for maintaining cellular homeostasis, primarily mediates the degradation of misfolded proteins in the endoplasmic reticulum (ER) through two pathways: ER-associated degradation (ERAD) and ER autophagy (ER-phagy). Tripartite motif 13 (TRIM13) is a protein located on the ER membrane, which plays a critical role in ERAD by its E3 ubiquitin ligase activity. In addition, TRIM13 also acts as a non-classical ER-phagy receptor to mediate the occurrence of ER-phagy. In recent years, TRIM13 has received extensive attention in the field of ERQC. This article reviews the structure and function of TRIM13, as well as the mechanisms by which TRIM13 contributes to ERQC, and summarizes its abnormal expression and regulatory role in diseases, with the aim of providing new strategies for the treatment of related diseases.