Mitochondrion, “the power house” of the cell plays a vital role in generating energy for the intricate functions of the cells. Mitochondria also play important roles in various apoptotic pathways. Around 80-90% of the ATP generated in cells is contributed by these organelles through the process of oxidative phosphorylation. Though this process is essential for the functioning of cells it also generates various Reactive Oxygen Species (ROS), which are toxic to cells. Hence mitochondrial dysfunction is hypothesized to be an important factor in the occurrence of disorders related to aging such as neurodegeneration and malignancies. Several commonly used drugs in clinical practice exert their action by interacting with mitochondrial pathways. This review attempts to focus on the various groups of drugs which act on mitochondria and are utilized for therapy of conditions like cancer, diabetes mellitus, neurodegeneration and so on.