Because of the age of onset, morphology and pluriphenotypic immunoprofile, the main differential diagnosis for SARC‐CIC is atypical teratoid and rhabdoid tumors (AT/RT), which are easily ruled out by immunohistochemistry (showing a retained expression of INI1 and BRG1). The gene discussed is SMARCB1; the disease is ataxia telangiectasia.