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.