A 73-year-old male presented with a slowly growing tumor in the right lower eyelid of one year's duration. The condition had been diagnosed elsewhere as poorly differentiated squamous cell carcinoma by biopsy. The residual tumor progressed rapidly and metastatized to the ipsilateral preauricular lymph nodes. We performed orbital evisceration and radical neck resection. Microscopically, the tumor showed small epithelial nests, cords, and ductal structure buried in abundant collagenous stroma. Besides focal squamous differentiation with keratinization in the surface area, the tumor showed, as cardinal features, ductal differentiation possessing comma-like extensions identical to syringoma. Cellular atypia, invasive growth pattern, and remarkable perineural invasion were suggestive of malignancy. These features led to the diagnosis of syringomatous carcinoma. Immunohistologically, the tumor showed positive staining for cytokeratine and epithelial membrane antigen (EMA). It was mostly negative for S-100 and carcinoembryonic antigen (CEA).