A 2 month-old baby was admitted to our emergency unit presenting hypoactivity, hyporexia, hepatosplenomegaly, hyperlipidemia, oliguria and generalized edema, compatible with congenital nephrotic syndrome. Additional investigation evidenced a reactive non treponemal test and her mother was untreated for gestational syphilis. No specific measure for nephrotic syndrome was taken, intravenous penicillin G was administered for 10 days with total remission of symptoms. Follow up after discharge confirmed that congenital syphilis was the cause of congenital nephrotic syndrome. Since syphilis case reports have been increasing worldwide, physicians should be aware of its uncommon manifestations.