Inflammatory pseudotumor-like follicular/fibroblastic dendritic cell (FDC/FRC) sarcoma is an extremely rare neoplasm of the spleen associated with EBV and characterized by spindle cell morphology, dense mixed chronic inflammatory background, and a broad immunophenotypic profile often causing a diagnostic challenge for pathologists. The molecular features of FDC/FRC sarcoma are largely unknown due to a lack of comprehensive data. Here we present the results of next-generation sequencing and Single Nucleotide Polymorphism Copy Number array analysis in a case of FDC/FRC and review the literature.