Henoch-Schönlein purpura (HSP) is the most common vasculitis among children, which its overall incidence rate is higher in boys than girls. This syndrome can affect skin, joints, gastrointestinal (GI) tract, kidney and, in rare cases CNS and scrotum. Genetic and environmental factors are considered to be possible triggers. HSP is typically self-limited, and most patients recover without therapy, however, severe intestinal bleeding or intussusception, intermittent hematuria, proteinuria and chronic kidney disease may occur in a small number of cases. In recent years, attempts have been made to identify genetic polymorphisms that may be associated with the development of HSP or with its severity. Many of these evaluated cytokines or cell adhesion molecules involved in the modulation of inflammatory pathways and endothelial cell activation. A few number of research work on a limited number of patients have been done and the results are varying among different ethnic groups. We review association studies on HSP to identify which genes or genetic polymorphisms significantly increase the risk of the disease.