A lethal case of Brazilian spotted fever (BSF) is presented. Clinical features were initially of gastrointestinal involvement and evolved with progression to septic shock, meningoencephalitis and death on the 6th day of illness. Indirect immunofluorescence assay (IFA) for spotted fever group rickettsia (SFGR) was non-reactive. Diagnosis was confirmed by the polymerase chain reaction (PCR) and the nucleotide sequencing of a fragment of the ompA gene showed 100% homology to Rickettsia rickettsii. BSF has not been reported in the city of Rio de Janeiro in the last three decades, and the present description should alert the clinicians to its presence in urban Rio de Janeiro, and to the differential diagnosis with dengue fever, gastroenteritis, leptospirosis and bacterial septic shock, among others. Key-Words: Brazilian spotted fever, spotted fever group rickettsia, Rickettsia rickettsii, lethal case, Rio de Janeiro city, indirect immunofluorescence, polymerase chain reaction, central nervous system involvement.