Follicular dendritic cell sarcoma (FDCS) is an extremely rare neoplasm which involves both nodal and extranodal sites. The demographics, pathological characteristics and clinical features of this tumor have been reported a lot, but the genomic information is still insufficient. Here we presented the ninth case of pulmonary FDCS in English publication, whose diagnosis has been revised from "synovial sarcoma" to FDCS. The patient underwent a right pneumonectomy and died of sudden cardiac death within a week after the surgery. Because of the lack of genomic characterization of the tumor, we used the OncoScan ® gene chip, which has been optimized for detection of whole genome CNVs. By bioinformatics' analysis and thoroughly literature review, we found the following suspicious functional genes REL, TET2, CREBBP and CYLD. These genes we found may have important influence on the fate of the neoplasm. Further study for their role of diagnostic and therapeutic targets is needed.