Most cases of aplastic anemia (AA) complicated by nephrotic syndrome (NS) have been reported to be the effects of chronic graft-versus-host disease after hematopoietic stem cell transplantation (HSCT). We describe a 16-year-old boy with AA with monosomy 7 who developed NS during immunosuppressive treatment for AA alone without HSCT. Recently, there appeared a report of MIRAGE syndrome caused by gain-of-function mutation of SAMD9 gene on chromosome 7 who developed NS. No such mutation was detected in our patient and thus the genetic factors leading to the complication of two diseases remain unknown at this time.