Mediterranean spotted fever (MSF) is a disease caused by Rickettsia conorii and transmitted by the brown dog tick Rhipicephalus sanguineus. It is widely distributed through southern Europe, Africa, and the Middle East. It is an emerging or a reemerging disease in some regions. Countries of the Mediterranean basin, such as Portugal, have noticed an increased incidence of MSF over the past 10 years. It was believed that MSF was a benign disease associated with a mortality rate of 1-3% before the antimicrobial drug era. It was called benign summer typhus. Severe forms were described in 1981, and the mortality rate reached 32% in Portugal in 1997. However, neurological manifestations associated with brain lesions are a rare event. We describe the case of a man with fever, maculopapular rash, a black spot, and hemisensory loss including the face on the left side of the body with brain lesions in the imaging studies.