This case series and literature review evaluated the baseline variables, clinical symptoms, pathological characteristics, and prognosis of patients with orbital oncocytic carcinoma.This retrospective case series collected the medical histories and other related data from 13 patients pathologically diagnosed with oncocytic carcinoma.The average age of patients with orbital oncocytic carcinoma was 64.8 years, with a significantly higher rate of males than females. Furthermore, unilateral disease was more common than bilateral disease. All patients had surrounding tissue invasion, most commonly to extraocular muscles (69.2%) and bones (53.8%). The clinical manifestations were proptosis (30.8%), swelling of the lesion area (23.1%), vision loss (23.1%), diplopia (23.1%), periocular mass (23.1%), tears (15.4%), eye pain (7.7%), ptosis (7.7%), and visual field loss (7.7%). Microscopic examination revealed many large eosinophilic cells. There were obvious nuclei and abundant mitotic figures. The cancer cells expressed cytokeratin, but not P63 or S-100. The follow-up duration was 2-53 months, and the metastasis rate was 16.7%. The patients exhibited a good prognosis. The main treatment methods included surgical resection, surgery combined with radiotherapy, and the enucleation of ocular contents.Orbital oncocytic carcinoma has the immunohistochemical characteristics of eosinophilic tumor cells, with expression of cytokeratin but not P63 or S-100. The prognosis is favorable. Surgical resection, surgery combined with radiotherapy, and enucleation of intraocular material are effective treatment options. Nevertheless, long-term follow-up and close observation for metastases are required.Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.