The immunoglobulin A (IgA) immunoregulation disorders lie at the basis of Henoch-Schönlein purpura nephritis and IgA nephropathy. Peliosis is the condition characterized by cystic formations within the parenchyma of solid organs filled with blood. The authors report a case of a girl presenting with hematuria occurring during the course of respiratory infections since her fifth year. Pathohistological examination was not performed at that time. At the age of 13, the girl was hospitalized for abdominal pain. Computed tomography examination showed the presence of multiple, relatively well-defined nodular formations located in the spleen parenchyma. Splenectomy was performed. Morphological finding completely corresponded to peliosis of the spleen, with the deposits of IgA in the lesions within it. A year and a half following the splenectomy, a typical clinical picture of Henoch-Schönlein purpura nephritis developed. Biopsy findings of the skin and kidneys detected deposits of IgA. This is the first case of a patient suffering from associated IgA and Henoch-Schönlein purpura nephritis complicated by splenic peliosis to be described in the world's literature.