Of 15 biopsy specimens identified as showing sarcoid involving the extraocular orbital tissues, three were from patients known to have systemic sarcoidosis before biopsy. Of the other 12 patients who were not known to have systemic disease at the time of biopsy, 11 were found to have evidence of systemic sarcoidosis after the biopsy. One patient, however, had no evidence of sarcoidosis at the time of the biopsy nor did she develop sarcoidosis during a seven-year follow-up period. Our results suggest that most patients with sarcoid involving the orbit will be found to have evidence of systemic sarcoidosis, but that rare patients exist in whom the orbital lesion appears to represent an isolated process. There was no histologic difference between orbital specimens from patients with systemic sarcoidosis and the specimens from the patient with isolated sarcoid of the orbit.