The etiology and pathogenesis of concomitant strabismus is complex. Some patients have a clear family history, indicating that genetic factors play an important role in the pathogenesis of concomitant strabismus. Concomitant strabismus is generally considered as a kind of disease with complex genetic pattern and high genetic heterogeneity. Esotropia and exotropia show different genetic pattern and heritability in different studies. In recent years, genetic studies revealed the role of 7q31.2 and 4q28.3 locus, variation of gene like ARIX, and NPLOC4-TSPAN10-PDE6G gene cluster in the pathogenesis of concomitant strabismus. Future studies need larger sample size, more pedigrees study, and more specific gene mutations. (Int Rev Ophthalmol, 2022, 46: 366-369)