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To report 2 patients with apocrine adenocarcinoma of the eyelid and review the literature.Noncomparative, retrospective case series and review.Clinical data of 2 patients with histopathologic diagnoses of apocrine adenocarcinoma of the eyelid were obtained from medical records. The histopathology and immunohistochemistry data for the 2 cases were studied. A retrospective review was performed on all reported cases of eyelid apocrine carcinoma.Clinical features, histopathology, immunohistochemistry, and clinical course following treatment are reported. The results of a retrospective review of clinical presentations, local and systemic spread, treatment method, and prognosis from published case reports on eyelid apocrine adenocarcinoma are discussed.One patient had orbital invasion at presentation. This patient had radiotherapy alone and had no systemic or local recurrence at 2 years. One patient underwent a 4-mm margin wide surgical excision. This patient had no local or systemic recurrences at 1 year. A review of the literature suggests that the prognosis of adnexal apocrine tumors is indeterminate and variable. Most patients have successful outcomes following local surgical excision. Radiotherapy has been used as an adjunctive treatment for local metastatic disease.Apocrine adenocarcinoma is a rare adnexal tumor of the eyelid, which may present with orbital invasion.