Congenital tuberculosis is a severe rapidly progressive disease which differs from neonatal tuberculosis because patients present tuberculous lesions during their first weeks of life, primary hepatic complex or hepatic caseous granulomas, confirmation of tuberculosis in placenta or in maternal genital tract (uterus or adnexa), and exclusion of birth channel or postnatal exposure. We report a case of a 20 days newborn baby admitted to the neonatal unit with fever, hepatoesplenomegaly, abdominal distension and respiratory symptoms. Abdominal echography showed ascitis, diffuse hepatomegaly and splenomegaly, focal hipoecoical spleen images and portal lymphadenopathy. Thorax X-ray with micronodular infiltrates. Maternal pneumonia with pleural effusion was reported during pregnancy. Empirical treatment was initiated with 4 antituberculous drugs: isoniazid, rifampicin, pyrazinamide and ethambutol suspecting tuberculosis. She died the next day. M. tuberculosis was obtained in a gastric lavage culture, blood cultures and post mortem spleen puncture.