Intra-abdominal desmoplastic small round cell tumor has been described in the literature since 1989. It is characterized by the occurrence in ages less than 40 with male predominance, an intra-abdominal location, and small round to oval shaped tumor cells with divergent differentiation in the background of the desmoplastic stroma. We recently experienced this tumor in an inguinal lymph node of a 36-year-old man. It is suspected that it metastasized from a lower intra-abdominal tumor. Immunohistochemical stains for keratin, epithelial membrane antigen, vimentin, S-100 protein and neuron specific enolase were positive. This is the first documented case in Korea. Herein, we report on this tumor with a review of literature.