In minimal-change steroid-sensitive nephrotic syndrome with selective proteinuria, mesangial IgA deposition at immunofluorescence is a very rare finding which has been previously considered a pure coincidence. Two patients, aged 6 and 14 years, respectively, with a steroid-sensitive but frequently relapsing nephrotic syndrome and highly selective proteinuria, exhibited minor glomerular alterations at light microscopy and an immunofluorescence deposition of predominant and diffuse mesangial IgA, confirmed by electron microscopy as dense deposits. The observed syndrome, that is surprisingly identical to sporadic literature reports, can be considered a separate entity or subgroup belonging either to IgA nephropathy or to lipoid nephrosis. In the latter case mesangial IgA could be the marker of an easy relapsing course.