Lymphatic malformations are benign hamartomatous tumors present at birth but usually diagnosed in early childhood. We report a case of prenatal diagnosis of an isolated unilateral retrobulbar lymphatic malformation with fetal magnetic resonance imaging (MRI). This was first detected at 27 weeks' gestational age. Postnatal ocular examinations at 4 days and 5 weeks of age showed no signs of optic nerve compromise. Postnatal MRI at 18 days of age showed slight increase in size of the lesion, and no intracranial vascular malformations were detected.
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