Amyotrophic lateral sclerosis (ALS) is the most common variant of motor neuron disease affecting adults that typically strike during mid to late life.The aetiology of this devastating condition, for which no cure has been developed, is poorly understood. A wide range of genes have been identified through the years which increase susceptibility to the disease, intensify the rate of motor neuron degeneration or are responsible for a given disease symptoms. One of the first and most widely studied gene, involved in pathogenesis of ALS, is Superoxide Dismutase–1 (SOD1). This gene encodes a Cu–Zn binding, anti–oxidizing enzyme that also has a role in extracellular signaling and apoptosis. Mutant SOD1 is associated with cellular pathology caused by protein aggregation, ER stress, Mitochondrial and axonal transport disruption. Potential of SOD1 as a therapeutic target for ALS is being actively researched with drugs that chelate excessively accumulated copper and RNAi strategies to reduce levels of mutant SOD1 being developed to combat this disease. This review deals with the possibilities of SOD1 as a prospective target to tackle this devastating condition.