The majority of purpura fulminans cases have an infectious etiology, but it is essential to consider other congenital and acquired causes.<h4>Case description</h4>We present a clinical case of a female neonate to emphasize the correlation between purpura fulminans, congenital chylothorax, involvement of the <i>PAK2</i> gene, and the occurrence of retinal detachment in both eyes. This evidence concerns the gene PAK2 and purpura.