Orbital lesions characterized by granulomatous inflammation are a heterogeneous group of diseases of various causes with a common histopathological substrate involving aggregates of epithelioid cells. Forty-one patients (27 females and 14 males) with biopsy-proven granulomatous inflammation were seen at an orbital clinic between 1978 and 1989. The mean age at presentation was 40.2 (extremes 6 and 77) years. Two main clinical presentations were noted: painless, subacute or chronic mass effect, and tender, subacute inflammatory process. Six patients had secondary features that were infiltrative in character. The lesions were primarily located in the anterior superior orbit. In nearly half the patients the granulomatous reaction was confined to the orbit (predominantly ruptured dermoid and localized orbital sarcoid), and the remainder had either regional involvement (Wegener's granulomatosis or fibro-osseous process) or systemic involvement (sarcoidosis).