Amyotrophic lateral sclerosis (ALS) is a fatal disorder of motor neuron degeneration with unclear etiology and no effective treatment to date. The cause of the most cases of ALS remains unknown. Research in ALS has been stimulated by a series of genetic and molecular pathology discoveries. ALS is believed to be a multifactorial and multi-system) disease. There is an intense need to establish biomarkers sensitive to diagnosis, prognostic stratification and disease activity of ALS. Based on this review, we mentioned about some biological markers according to the genetic and molecular pathology reports; in future studies these all may guide us.