Neonatal purpura fulminans is a rare disease. Diagnosis is a challenge during the first days of life. We report a newborn who presented distal ecchymotic lesions with rapid progressive extension in the first hours of life. A deficiency of protein C or S was suspected. The genetic study reported two variants of the PROC gene (c.303C>A; c.400+5G>C) confirming the diagnosis of congenital deficiency of protein C. Supportive therapy with fresh frozen plasma and anticoagulation with low molecular weight heparin was provided. During follow-up, the patient presented amputation of the affected limbs, retinal detachment and cerebral ischemia. Congenital protein C deficiency requires timely diagnosis, and specialized management is important to prevent complications of this disease.