IgA vasculitis (IgAV) or Henoch-Schönlein' s purpura is the most prevalent systemic small vessel vasculitis in childhood. High mobility group box-1 protein (HMBG1) is a pleiotropic cytokine that functions as a pro-inflammatory signal, important for the activation of antigen- presenting cells (APCs) and propagation of inflammation. HMGB1 is implicated in the pathophysiology of a variety of inflammatory diseases. The aim of this study was to investigate the role of single nucleotide polymorphism (SNP)- rs41369348 for HMGB1 gene in the susceptibility and clinical features of patients fulfilling classification criteria for IgAV. DNA was extracted from blood cells of 76 children with IgAV and 150 age-matched healthy controls. Clinical data and laboratory parameters were collected for all IgAV patients. Although there was higher frequency of heterozygous A/delA genotype of this gene polymorphism in IgAV group compared to control group, no genotype difference between those two groups was observed. No statistically significant differences in genotype were disclosed when patients with different IgAV clinical features were compared. In conclusion, in this study polymorphism rs41369348 for HMGB1 was not associated with increased susceptibility to childhood IgAV, nor with its severity or different clinical manifestations.