We report the case of a 17-year-old male patient with constant alteration of the prothrombin time and episodes of self-limited macrohematuria. After several studies we found out a deficiency of Factor X levels, whereas all other coagulation factors levels were normal. We continued with the study of the hematuria with urine and blood analysis, imaging tests and viral serologies, which finally made it possible to rule out any other primary or secondary glomerulopathies and to focus on IgA nephropathy as the most likely diagnosis. In this release we explain in detail the diagnostic tests performed. We have also reviewed the two entities separately and together, in search of a genetic disease which associates both pathologies. Palabras clave: Hematuria. Déficit de Factor X. Nefropatía por IgA.