A 30-year-old female had been suffering from multiple pruritic nodules on her extremities since elementary school. Her local doctor prescribed topical steroids for external use as needed. An ulcer that was refractory to oral antibiotics and isodine sugar paste ointment appeared on the right leg 7 months before her rst visit to our department. At the initial examination, a necrotic and cavelike ulcer, 3 cm×3 cm in size, satellited with old pruritic nodules was observed. Skin biopsy from the border of the ulcer revealed a ower-basket-like proliferation of spindle-shaped cells in the dermis which were strongly positive for CD34 ; therefore, the initial diagnosis was dermatobrosarcoma protuberans. A second histopathological examination after wide resection of the tumor revealed epithelial-like oval cells in addition to spindle-shaped cells deeply proliferated from the dermis to muscular fascia with an island-like pattern structure. Based on these immunohistochemical and histopathological results, the tumor was diagnosed as epithelioid sarcoma. We performed lymph node dissection for inguinal lymph node metastasis. Epithelioid sarcoma is a rare soft tissue malignancy that is prone to local recurrence and distant metastasis. Our ndings suggest that although epithelioid sarcoma is rare, it should be considered a differential diagnosis for intractable skin ulcer. Skin Research, 22 : 19-23, 2023