Schimke immuno-osseous dysplasia (SIOD) is a very rare, genetically determined disease associated with SMARCAL1 gene mutation. SIOD is characterized by multisystem disorders with progressive nephropathy, skeletal, immunological, hematological and dermatological abnormalities, short stature and dysmorphia. In this study, the case of a boy with nephropathy in the course of SIOD was presented. At the age of 3 years, proteinuria with coexisting hypertension occurred. Histopathological examination showed minimal change in the disease. At the age of 6 years, the progression of nephropathy to primary steroid resistant nephrotic syndrome was observed. After the institution of cyclosporine A therapy, the nephrotic syndrome entered remission. Over a 6-year follow-up the patient presented with episodes of subnephrotic proteinuria and normal renal function. At the onset of follow-up, among the extrarenal findings, intrauterine growth restriction occurred and at the age of 3 years, leukopenia was diagnosed. In the following years, other clinical manifestations such as short stature, typical dysmorphic features, pigmented macules and spondyloepiphyseal dysplasia were revealed. Genetic examination revealed SMARCAL1 gene mutation. In conclusion, nephropathy may be the first clinical feature of SIOD. In contrast to the majority of cases, in our patient the nephrotic syndrome was due to minimal change disease. In addition, cyclosporine A therapy was successful and renal function remained normal during follow-up over a few years.