A rare lymphoproliferative disorder involving thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O), so called TAFRO syndrome, was first reported in 2010.1 Considered a variant of idiopathic multicentric Castleman’s disease, the recent discovery and rarity of this syndrome poses significant challenges to diagnosis and management. In this case report, we review the youngest patient to be reported in the literature with TAFRO syndrome. We highlight the patient’s diagnosis, management, and follow-up in the context of current recommendations for treatment and management of TAFRO syndrome.