Paragangliomas of the orbit are extremely rare. We report on a case of paraganglioma manifesting as enlargement of the lateral rectus muscle. Magnetic resonance imaging (MRI) of the orbit showed typical salt and pepper appearance of the mass and pathologic examination was consistent with paraganglioma. The patient underwent surgery with total removal of lateral rectus muscle. Alignment was preserved by a half tendon transposition of the vertical rectus muscles to the insertion of the resected lateral rectus muscle. Isolated lateral rectus enlargement has not been previously reported as a manifestation of paraganglioma.