Protein C is a vitamin K dependent glycoprotein. Protein C deficiency is a rare genetic disorder and its major sites of involvement are skin, eyes, lungs, central nervous system, and kidneys. This article presents a case of neonatal protein C deficiency with severe purpura fulminans and bilateral cataracts. He was initially treated with fresh-frozen plasma and then followed by warfarin. All necrotic skin lesions improved with treatment. He was the first child of his parents who were asymptomatic for protein C deficiency. We also reviewed literatures about coexistence of cataracts and protein C deficiency.