Dendritic cell neoplasms are rare malignancies described with an increased frequency. They often involve the lymph nodes, but extranodal presentations are also described. The disease often has an indolent course. Surgery is the main modality though chemotherapy and radiation have also been tried. We present here a case of a 69-year-old male diagnosed to have follicular dendritic cell sarcoma of the neck which had a rapid and fatal course inspite of chemotherapy and radiotherapy. More studies are required to assess the biology of this rare tumor.