We report a case of erythema annulare centrifugum (EAC) with neonatal onset. The patient presented on the fourth day of life with erythematous papules that enlarged centrifugally to form annular or policyclic plaques on anterior surface of trunk and legs without signs of systemic toxicity. Subsequently, she developed sepsis and disseminated intravascular coagulation and died on the sixth day of life. Blood culture grew Pseudomonas aeruginosa. The coexistence of P. aeruginosa sepsis with the clinical appearance of erythema annulare centrifugum strongly suggests a pathogenetic effect of the microorganism on the skin eruption. To the best of our knowledge, this is the first report of EAC with P. aeruginosa sepsis in a newborn.