Coenzyme Q (CoQ), also known as ubiquinone, is an essential lipophilic molecule present in all cellular membranes and involved in a variety of cellular functions, in particular as an electron carrier in the mitochondrial respiratory chain and as a potent antioxidant. CoQ is synthesized endogenously through a complex metabolic pathway involving over 10 different components. Primary CoQ 10 deficiency in humans, due to mutations in genes involved in CoQ biosynthesis, is a heterogeneous group of rare disorders presenting severe and complex clinical symptoms. The generation of mouse models deficient in CoQ is important to further clarify the cellular function of CoQ and to unravel the complexity in the pathophysiological consequences of CoQ deficiency. This review summarizes the current knowledge on mouse models of primary CoQ deficiency.