The present study describes clinical manifestation, histopathologic findings and differential diagnosis of a first-reported case of giant chondroid syringoma (CS) of the upper nasal orbital rim. A 35-yr-old man with cranial palsy (CP) and a tumor of 3×3×4 cm in the upper nasal orbital rim of the left eye was referred to the eye clinic. The tumor was reported to have developed gradually over the years. It was removed under general anesthesia and no recurrence was observed during the one-year follow-up without any complaint from the patient. Macroscopic examination showed a whitish, irregular and relatively soft nodule of 3×2.5× 2 cm. and with calcificated spots on its section. Microscopically, tubules of various sizes were observed which were surrounded by cellular stroma and distinct areas of chondroid proliferation with no malignant changes. CS may assume a giant size, appearing as hard, slow-developing cutaneous and subcutaneous nodules on differential diagnosis. Despite favorable prognosis, the recurrence of CS is reported only in those patients whose tumors are not completely removed.