A 74-year-old Japanese woman presented to our hospital with multiple ulcerated nodules on the left hand and forearm. The nodules had slowly increased in size and number for 2 months. An examination revealed a 2-cm ulcerated nodule on her left hand; she had multiple satellite lesions on her left hand and forearm. The initial differential diagnoses included subcutaneous mycosis and malignant melanoma. Histopathological examination showed ulceration and granulomatous lesions with multinucleated giant cells in the deep dermis. A periodic acid-Schiff stain revealed no fungi in the specimen, but a tissue culture was positive for Alternaria, a dematiaceous fungus. A diagnosis of dermal cutaneous alternariosis was made. The patient was started on itraconazole therapy, but her nodules increased in size and number within the next 2 months. Rebiopsy of a nodule on the patient’s palm revealed the proliferation of atypical polygonal or oval epithelioid cells in the dermis and lymphocyte infiltration through the dermis. Immunohistochemically, the tumor cells were positive for vimentin, cell adhesion molecule 5.2 (CAM5.2), epithelial membrane antigen (EMA), and E26-related gene (ERG); they were negative for AE1/AE3, CD34, S100, smooth muscle actin, and CD31. The nuclear expression of integrase interactor 1 (INI1) was lost in the tumor cells. On the basis of these findings, a diagnosis of ES was made. Preoperative fluorodeoxyglucose-positron emission tomography showed elevated glucose levels in the multiple lesions on the patient’s left hand, left forearm, and left axillary lymph nodes. After shoulder disarticulation and axillary lymph node dissection, there was no recurrence, and no metastases were observed during 9 months of follow-up.