Superficial epithelioma with sebaceous differentiation is the original name for a rare benign tumor that lacks consensus in the literature, both in nomenclature and in diagnostic criteria. We report the case of a 68-year-old male with a white papule on the left lower eyelid, fully excised. On histology, the lesion was a superficial, lobulated, plate-like epithelial proliferation, with small mature clusters of sebocytes and ductal structures. Mismatch repair proteins MLH-1, MSH-2, MSH-6, and PMS-2 were retained in the immunohistochemistry study. We reviewed the literature and added our case, which we believe contributes to better characterization of superficial epithelioma with sebaceous differentiation. It usually presents as a solitary papule or nodule on the face of middle-aged males. Histologically, besides the hallmark of a superficial plate-like epithelial proliferation, some variations are occasionally reported: the presence of randomly scattered keratin-filled cysts, squamous eddies, minimal peripheral palisading, marked verruciform architecture, and a reticulated pattern with elongated strands of keratinocytes. The mitotic activity can be high, but no cytological atypia or necrosis is seen. If properly removed, there is no evidence of local recurrence or distant spread. The association with Muir-Torre syndrome remains unclear.