Follicular dendritic cell (FDC) sarcomas are rare tumours, typically seen in lymph nodes. However, in about one third of the reported cases, a FDC sarcoma presents as an extranodal mass. Involvement of the gastrointestinal tract is extremely rare, and only 3 cases have been described to date. We report on a 40-year-old female patient with a follicular dendritic cell sarcoma located in the stomach and the presence of a metastasis in the liver at the time of diagnosis. Severe asthenia, nausea, back pain and loss of weight were the presenting symptoms. A CT scan of the abdomen and an upper gastrointestinal endoscopy revealed a tumour mass in the stomach. The diagnosis of a FDC sarcoma was made on histological and immunohistochemical findings. We report the second case of a FDC sarcoma presenting in the stomach. Due to its rarity, a FDC sarcoma seldom enters the differential diagnosis of spindle cells neoplasms of the gastrointestinal tract. Complete surgical resection is the treatment of choice for FDC sarcoma.