Neurofibromatosis type I (NF-1), the most common neurocutaneous disorder, can be an inherent or spontaneous mutation of the NF-1 gene on chromosome 17q11.2 and encodes neurofibromin proteins. There have only been a few cases reported of NF-1 associated with nephrotic syndrome and the relation rests unclear. Herein, we present a case of NF-1 combined with minimal change disease (MCD).