Abstract: Mitochondrial autophagy refers to the selective autophagy to remove dysfunctional or excessive mitochondria in order to maintain the stability of intracellular homeostasis. The Pink1/Parkin signaling pathway is most widely studied in mitochondrial autophagy-related pathways. In mammalian cells, the Ser/Thr kinase Pink1 and the E3 ubiquitin ligase Parkin synergistically act to sense mitochondrial status and tag damaged mitochondria for autophagic degradation through autophagy pathways. Meanwhile, ubiquitination and deubiquitination enzymes play important roles in regulating Parkin activity and efficiency of mitochondrial autophagy. In this review, the Pink1/Parkin signaling pathway and the roles of deubiquitination enzymes in mitochondrial autophagy are discussed.