Congenital Orbital Teratoma with Unilateral Proptosis

Teratoma is a rare tumor, containing structures originating from all three germinal layers. The most frequent location of teratoma is the gonads. Orbital teratoma is extremely rare. In 1969, Jensen reported that only 40 cases of orbital teratoma existed in the world. We describe a rare case of orbital teratoma in a 15-day girl who presented with marked proptosis of the left eye. The eyeball was embedded within the mass that could be seen all around the globe. Orbitectomy was performed with the impression of retinoblastoma. Histopathological examination was reported as benign mature cystic teratoma; no immature component was identified. The case is being reported because of its rarity and also to highlight the use of modern neuroimaging techniques in making an accurate preoperative diagnosis, which helps in better operative management of these patients.