We present a 21-year-old man with common variable immunodeficiency (CVID) and a phenotype of immune dysregulation similar to CTLA-4 haploinsufficiency characterized by autoimmune cytopenia, inflammatory bowel disease (IBD), lymphoproliferation (GLILD), autoimmune encephalitis, and humoral deficiency requiring multiple immunosuppressants (Figure 1). This evidence concerns the gene CTLA4 and inflammatory bowel disease.