ALS is a progressive neurodegenerative disease. It is characterized by motoneuron degradation which leads to muscle atrophy and death due to respiratory failure. The only drugs approved for ALS treatment are riluzole and edaravone, a benzothiazole modulating glutamate uptake and an antioxidant, respectively. Research conducted in the last decades identified neuroinflammation as one of the main ALS hallmarks. It is characterized by microglial activation, infiltration of T cells and astrocytosis. T cells are present in negligible numbers in the healthy central nervous system (CNS) but cross the blood-brain-barrier at the site of injury and are thought to activate microglia and promote motor neuron damage. Microglial and astroglial activation furthers this process. Mutations found in genes encoding for SOD1 (superoxide dismutase), FUS (RNA-binding protein FUS), TDP-43 (TAR DNA-binding protein 43) and C9orf72 (chromosome 9, open-reading frame 72) have been found to indirectly or directly modulate the immune system. Neuroinflammation is thought to be an aftermath of disrupted autophagy and/or hyperactivation of the NF-κB pathway, which in turn result in an increased release of pro-inflammatory cytokines from microglia. All these findings open a possibility that immunomodulatory therapies could be a promising approach in ALS. Therefore, some of the therapies focus on immune response manipulation as a means of decreasing the inflammation and blocking the disease progression. This thesis will present major genetic mutations found in ALS patients and explain their connection with the immune system, supporting the hypothesis that immunomodulation is a promising therapeutic approach.