Members of the AAA+ (ATPases associated with diverse cellular activities) superfamily, contribute to a wide variety of cellular processes. They play important roles in membrane fusion, organelle and protein trafficking, DNA replication, protein disaggregation and protein degradation. In mammals, several different AAA+ proteins have been identified in the mitochondrion. Although most of these proteins contribute to protein homeostasis or the maintenance of respiratory competence in mammalian mitochondria, the role of one mitochondrial AAA+ protein (SKD3) remains unknown. SKD3 is a unique member of the AAA+ superfamily. It contains a single AAA domain which is most closely related to the D2 domain of ClpB and Hsp104, both of which function in protein disaggregation. However, in contrast to the ClpB/Hsp104 family of proteins, SKD3 contains four N-terminal ankyrin repeats and lacks the coiled-coil middle domain, which is essential for protein disaggregation. As such it is unclear what role SKD3 may play in mammalian mitochondria. Here we will present our current view of SKD3 function in human mitochondria.