Follicular dendritic cell sarcoma is a rare malignancy arising from follicular dendritic cells, which form a meshwork within lymphoid follicles. Traditional treatment of metastatic disease with CHOP chemotherapy, the most commonly used regimen for lymphoid malignancies, has met with limited success. I report herein two cases of follicular dendritic cell sarcoma metastatic to the liver treated with the combination of gemcitabine and docetaxel showing unprecedented response in this disease. These observations coupled with recent evidence of a mesenchymal origin of follicular dendritic cells support the premise that this malignancy should be treated similar to other adult soft tissue sarcomas.