Superficial Ewing sarcoma of the skin and dermis is extremely rare and may closely simulate benign adnexal tumors or neuroendocrine carcinomas, leading to major diagnostic pitfalls. We report the case of a 19-year-old woman presenting with a painful, rapidly enlarging 1.5 cm nodule at the cervico-occipital region. Histologically, the lesion exhibited a compact multilobulated architecture within the dermis, with a striking “blue ball in dermis” appearance suggestive of spiradenoma. Immunohistochemistry was misleading, showing focal dot-like AE1/AE3 staining, heterogeneous low-to-moderate synaptophysin and chromogranin expression, CD56 positivity, and CK20 dot-like staining, raising the possibility of Merkel cell carcinoma. Strong diffuse CD99 positivity and nuclear NKX2.2 expression supported a Ewing sarcoma family tumor. Molecular analysis confirmed an EWSR1 rearrangement. This case highlights the diagnostic challenges of superficial small round blue cell tumors and emphasizes the need for integrated histologic, immunohistochemical, and molecular assessment.