Granular cell tumor of the orbit

To report a case of granular cell tumor as a rare orbital pathology. A 50-year-old female presented with a 4-year history of diplopia, right ocular displacement and a firm nontender mass in her right lower lid. Computed tomography (CT) scan of the orbit disclosed a well-defined mass in the right inferior orbit involving the right inferior rectus. Subtotal excision of the mass was performed, and histopathologic and immunohistochemical studies revealed granular cell tumor. Subsequently, the tumor recurred and exenteration was required as multiple sessions of radiotherapy failed to prevent the residual tumor from growing. Granular cell tumor, though very rare in the orbit, should be considered in patients with orbital masses especially in cases with involvement of the inferior rectus muscle. Infiltrative tumors may be impossible to completely resect and can rapidly recur following surgery.