A 25-year-old female presented with progressive swelling at the right external auditory meatus and bleeding from the mass when a crust was removed from its surface. Examination revealed a round ulcerated, skin-covered mass located on the posterior-superior wall of the entrance of the EAC, which partially obstructed the canal (
Fig. 1A). The medial EAC, eardrum and an audiogram were normal. Contrast-enhanced computed tomography (CT) showed immensely enhanced round mass with no invasion of adjacent structures and normal middle ear cavity (
Fig. 1B). These findings strongly suggested that the tumor originated from vascular tissue. Four-vessel angiography showed that it was fed by the posterior auricular artery, which was embolized successfully (
Fig. 1C, D). Under general anesthesia, the tumor was excised under an operating microscope; there were no adhesions to the surrounding tissues. The defect in the EAC was covered with a full-thickness retroauricular skin graft. The surgical specimen measured 2.0×1.8×1.5 cm with attached skin (1.8×1.0 cm). On cut section, the tumor was a well-demarcated, non-encapsulated, solid, gray-white nodule in the dermis (
Fig. 2A). There was no necrosis or hemorrhage. Histologically, the mass comprised predominantly spindle cells of moderate cellularity in a patternless architecture of hypo- and hypercellular areas separated by thick, hyalinized collagen with occasional vascular clefts (
Fig. 2B) with focal myxoid stroma. The tumor cells had minimal nuclear pleomorphism (
Fig. 2C). Roughly two mitotic figures were found per ten high-power fields, but no atypical mitotic figures were identified. Immunohistochemically, tumor cells showed a positive reaction for CD34, and partially positive for bcl2 (
Fig. 2E) (×200) and CD99 (
Fig. 2F) (×200). (
Fig 2D, F) But they did show a negative reaction for cytokeratin, epithelial membrane antigen, S-100 protein, smooth muscle actin, CD68 and CD31. The Ki-67 proliferative index was 2%. Based on these findings, it was diagnosed as a SFT.