Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare subtype of dendritic cell neoplasms, and current knowledge on this tumor is limited. We herein report a case of an IDCS in a 64-year-old man who presented with a right inguinal mass combined with extensive retroperitoneal, pulmonary, hepatic, renal, and bone marrow infiltration. Because of the advanced stage of the disease, we performed five cycles of chemotherapy, including cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP); doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD); and ABVD combined with cisplatin, and one cycle of radiotherapy. The patient's inguinal mass became smaller during the treatment, but there was no change in the extent of infiltration at the other sites. The patient died 8 months after the initial diagnosis. We also herein review the etiology, diagnosis, differential diagnosis, treatment, and prognosis of IDCS, and analyze the characteristics of IDCS in Chinese patients.