Background: The occurrence of proptosis at birth is unusual and may be
associated with a variety of rumours and structural abnormalities. Co
ngenital orbital teratoma is a rare cause of congenital proptosis. Met
hods: A case report is presented of a female infant with gross right p
roptosis. Results: Computed tomography demonstrated a characteristic m
ulticystic structure with no intracranial involvement Histological exa
mination found tissues derived from all three germ cell layers, consis
tent with a congenital orbital teratoma. The tumour was successfully r
emoved preserving the globe and vision. Conclusions: This report, is a
n example of how early surgical intervention may allow preservation of
the globe and vision in some patients with congenital orbital teratom
a.