A case of congenital orbital teratoma with marked unilateral proptosis was noted at birth, Patients with congenital orbital teratoma had a uniform clinical picture and were very similar in their morphological pattern, often polycystic and with varied amounts of solid tissue. Six months after birth, the orbital tissues were totally extirpated. Histological examination revealed components from all three germinal layers and no sign of malignancy. Within the first year, the condition of the patient has been satisfactory.