Abstract: Inflammatory Bowel Disease (IBD) is a chronic relapsing intestinal inflammation with no curative therapy currently available, primarily encompassing ulcerative colitis (UC) and Crohn's disease (CD). Its pathogenesis is multifactorial, involving genetic predispositions, environmental triggers, microbial dysbiosis, and immune dysregulation. The global prevalence of IBD is increasing continuously, imposing a substantial disease and economic burden worldwide. Mitochondria, serving as the cellular hub for energy production and signal transduction, are crucial for maintaining intestinal homeostasis and health through their dynamic processes and quality control mechanisms. A growing body of evidence indicates a definitive association between the onset and progression of IBD and mitochondrial dysfunction. A systematic review of IBD and its association with mitochondrial dysfunction contributes to elucidating the specific mechanisms of mitochondria in the pathogenesis and progression of the disease, thereby providing a crucial foundation for exploring novel therapeutic targets and preventive strategies.