An 84-year-old man presented with a 2-year history of a progressive left-sided ptosis. Examination demonstrated a mechanical ptosis and concentric constriction of the palpebral aperture. CT imaging revealed demonstrated diffuse soft tissue infiltration of the upper and lower eyelids with extension into the anterior orbit. This case was diagnostically challenging because of a history of multiple other primary tumors. However, clinicoradiologic and histopathologic findings were consistent with a diagnosis of primary adnexal signet-ring cell/histiocytoid carcinoma. The patient underwent surgical excision but local recurrence was noted 2 months postoperatively.