The TNFAIP3 gene encodes a zinc finger protein and ubiquitin-editing enzyme, and variants in TNFAIP3 have been associated with autoinflammatory syndrome (OMIM #616744) characterized by hemolytic anemia, thrombocytopenia, autoinflammation, and autoimmune lymphoproliferative syndrome, the latter presenting with lymphadenopathy and hepatosplenomegaly (23). Here, TNFAIP3 is linked to Hepatosplenomegaly.