The authors describe a 23-year-old man with unilateral upper eyelid swelling that evolved into a multinodular lesion with central necrosis, mimicking a neoplasm. Biopsy showed a lympho-histiocytic, eosinophil-rich proliferation with positivity for Langerhans cell markers CD1a and S-100 and histiocytic marker CD68. A literature review disclosed 11 documented cases of Langerhans cell histiocytosis of the eyelid with variable clinical presentations. This rare eyelid lesion, nearly always solitary, has no clinically distinctive characteristics and requires biopsy for diagnosis. Langerhans cell histiocytosis (LCH), formerly histiocytosis X, comprises a group of rare disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells intermingled with mature eosinophils. Proliferations may be multifocal (disseminated) or unifocal (solitary). Ophthalmic lesions are usually unifocal and tend to be located in the orbital region. Isolated eyelid foci are rare and present with variable patterns that preclude clinical diagnosis. In this report, the authors describe a case of multinodular, ulcerative involvement of the eyelid skin that mimicked a basal cell carcinoma. This study was conducted in compliance with the rules and regulations of the Health Insurance Portability and Accountability Act.