SMARCB1 is a member of the SWI/SNF chromatin remodeling complex and regulates transcription of several genes involved in cell proliferation. SMARCB1 mutations are rare in colorectal adenocarcinomas and are reported in only about 1% of cases. Although not biochemically assessed, SMARCB1 R377H has been identified as a statistically significant hotspot and is predicted to be oncogenic. The clinicopathologic significance of SMARCB1 variants in colorectal cancer remains to be further elucidated.