Acral myxoinflammatory fibroblastic sarcoma is a rare low-grade malignant soft tissue tumor, usually observed in the extremities of middle-aged patients. We report a case involving the third finger of the left hand of a middle-aged man. The tumor showed a nodular architecture, with cellular areas, occasional foci of hyalinized fibrosis, and hypocellular areas with a myxoid background. Various neoplastic cells were identified including spindled or rounded epithelioid cells and occasional bizarre giant cells, morphologically mimicking ganglion cells. Tumor cells were strongly immunoreactive for vimentin and variably positive for CD68 and CD34. The tumor was completely removed, without further treatment.