This case re-emphasizes a common origin of these entities, PPV and KTW, from the neural crest cells. Early recognition of the systemic features and timely surgical intervention under appropriate precautions, can be vision salvaging in such cases of developmental glaucoma.How to cite this article: Singh K, Dangda S, Mutreja A, Bhattacharyya M, Jaisingh K. Bilateral Phacomatosis Pigmentovascularis in a Young Male with Developmental Glaucoma and Varicose Veins. J Curr Glaucoma Pract 2018;12(2):94-98.