An 8‐year‐old boy with a heterozygous TGFBR2 (NM_003242.6):c.1583G>A (p.Arg528His) variant, genetically diagnosed as LDS, was referred to our department due to bloody stool, abdominal pain, and atypical endoscopic findings for IBD. This evidence concerns the gene TGFBR2 and inflammatory bowel disease.