Childhood orbital teratomas are congenital lesions that presents most often at birth with progressive, severe unilateral proptosis. Due to the rarity of such tumors, the diagnosis is often missed with delay in the patient's management. We are presenting a unique case of an immature right orbital teratoma with extensive growth in a full-term newly born baby boy. In this case report, we provide description of the clinical findings, initial misdiagnosis and the eventual management with review of similar reported cases.