Although major pathological conditions in FA are outcomes of the defects in DNA damage and repair machinery, recent findings suggest that bioenergetic pathways are compromised as well. A few FA proteins have functions that include redox balance, apoptosis, and energy metabolism; and recent data have established respiratory defects in FA cells, proposing that mitochondrial dysfunction (MDF) is involved in FA. This data was further supported by the finding that FA cells have elevated levels of mitochondrial ROS, decreased Mitochondrial Membrane Potential, decreased ATP production, impaired oxygen uptake and changes in the morphology of mitochondria. This was also accompanied by inactivation of enzymes that are essential in the energy production pathway. These findings suggest that mitochondrial activity and its metabolism may be a point of convergence in the pathological manifestations of FA. Mitochondrial dysfunction may thus be considered as an important phenomenon in FA which might account for most of the findings observed in FA's clinical phenotype. Exploring MDF in FA will provide new facets to the development of diagnostic and therapeutic strategies for FA. © 2015 by Nova Science Publishers, Inc. All rights reserved.