The incidence of congenital nephrotic syndrome secondary to congenital syphilis is very uncommon inclinical practices these days. We present a case of a two-month-old girl, who admitted with fever, cough,distended abdomen. The laboratory informed normal kidney function with Albumin 11.9 g/l; Protein 43.6 g/l;Cholesterol:1.64 mmol/l, Proteinuria 2.8g/l, urine Protein/Creatinin = 10g/mmol , VDRL (+), TPHA (+).Abdominal ultrasound showed ascitis and hepatosplenomegaly. The patient was diagnosed as congenitalnephrotic syndrome secondary to congenital syphilis. She was prescribed Albumin infusion, Diuretics,Penicillin. She presented good evolution and the syndrome was solved after 2 weeks of treatment