BACKGROUND. Our patient presented with an unusual case of rapidly growing palpebral tumors, which obstructed his vision and caused professional and social impairment. OBJECTIVES. The objective was to report the role of surgical management in a case of the d'emblee variant of cutaneous T-cell lymphoma and to provide a literature review. METHODS. Tumors of the eyelids previously treated with topical and oral retinoids, indomethacin, dapsone, systemic chemotherapy, and high-dose systemic corticosteroids respond finally to conservative excision and second intention healing. RESULTS. Surgical debulking of palpebral tumors achieved remission of a locally aggressive form of cutaneous T-cell lymphoma and significant restoration of the sight, eyelid function, and social/professional function. CONCLUSION. In selected cases, surgical excision of tumor tissue may be of significant assistance in managing d'emblee variant cutaneous T-cell lymphoma.