Background: The occurrence of proptosis at birth is unusual and may be associated with a variety of tumours and structural abnormalities. Congenital orbital teratoma is a rare cause of congenital proptosis.
Methods: A case report is presented of a female infant with gross right proptosis.
Results: Computed tomography demonstrated a characteristic multicystic structure with no intracranial involvement. Histological examination found tissues derived from all three germ cell layers, consistent with a congenita orbital teratoma. The tumour was successfully removed preserving the globe and vision.
Conclusions: This report is an example of how early surgical intervention may allow preservation of the globe and vision in some patients with congenital orbital teratoma.