schimke immuno osseous dysplasia is a rare autosomal recessive multisystem disorder characterized by steroid resistant nephrotic syndrome immunodeficiency and spondyloepiphyseal dysplasia mutations in swi snf2 related matrix associated actin dependent regulator of chromatin subfamily a like 1 smarcal1 gene are responsible for the disease the present report describes for the first time a schimke immuno osseous dysplasia child with smarcal1 missense mutation r561h and manifestations of intussusception secondary to epstein barr virus negative non hodgkin lymphoma who expired due to septicemia following chemotherapy the report emphasizes the necessity of more limited immunosuppressive protocols in schimke immuno osseous dysplasia patients with lymphoproliferative disorders.