Orbital Sarcoidosis: A Brief Guide to an Insidious Disease

Sarcoidosis is a rare systemic disease that can affect any organ. Orbital manifestations constitute an uncommon extrapulmonary form of sarcoidosis. The symptoms and imaging findings can mimic those of other pathologies, such as orbital pseudotumor lesions, thus delaying clinical diagnosis and adequate treatment. We systematically reviewed the existing literature on the topic, published in PubMed and Google Scholar, up to August 2025, regarding the etiology, clinical presentation of the disease, diagnostic methods, and management. Search words included "orbital sarcoidosis," "sarcoidosis/"orbital," and variations of these terms. Only English-language articles were selected. All broader articles that dealt with ocular sarcoidosis or neurosarcoidosis were excluded. The literature review is based on 18 articles (case reports, case studies, and original articles). It presents the current data for orbital sarcoidosis in five relevant sections: a brief review of the relevant anatomy of the orbit, presentation, diagnostic methods and evaluation, management of the disease, differential diagnosis, and related diseases. Sarcoidosis remains a challenging pathology to diagnose and manage. Its orbital manifestation is an insidious imitator that can undermine timely treatment and lead treating medical professionals to a false diagnosis. Along with the nonspecific clinical presentation, orbital sarcoidosis can be a peculiar disease that goes unnoticed for a long period.