Congenital orbital teratoma is a rare benign tumor, composed of all three germ cell layers. The Lesion presents clinically as uniLateral proptosis in the newborn. In order to diagnose the tumor correctly a multidisciplinary approach is needed, including ophthalmologists, neurosurgeons, pediatrics, radiologists, and pathologists to eventually diagnose the lesion. Early detection and treatment is needed in order to prevent mechanical destruction of adjacent tissues, and blindness from mechanical pressure on the optic nerve. Surgical excision is the treatment of choice. We present a case report of a newborn, diagnosed with congenital orbital teratoma, and discuss the clinical and histological characteristics of the tumor.