We report a case of a superficial angiomyxoma with a trichofolliculoma, found on the trunk of a 51-year-old man. The tumor, measuring 4 cm in maximum diameter and located in the reticular dermis and subcutaneous tissue, was composed of a conglomerate of multiple myxomatous nodules with mucinous stroma and stellate cells. Thin-walled blood vessels were prominent. The histologic examination also revealed abortive hair follicles radiating from the wall of a keratin-filled cyst. Immunohistologically, the stromal cells were positive for vimentin but negative for S-100, smooth muscle a-actin, and desmin. With these findings, the diagnosis of superficial angiomyxoma with trichofolliculoma was made.