Lymphatic malformations are relatively uncommon orbital lesions which are usually diagnosed in early childhood. Their exact etiology is unknown. Persistent Hyperplastic Primary Vitreous (PHPV) results from the failure of the embryological primary vitreous and hyaloid vasculature to regress. We report a rare case of a 9-year old male child who presented to our clinic with unilateral coexisting superficial orbital lymphatic malformation with persistent fetal vasculature manifesting as visual compromise and leukocoria.