Mediterranean spotted fever (MSF) is an infectious disease endemic in the southern regions of Italy, with an incidence of about 400 cases/year. The bacteria responsible of the disease is Rickettsia conorii, transmitted to humans by Rhipicephalus sanguineus, the common dog tick. The infection usually manifests with a characteristic symptomatologic triad: fever, exanthema and the so called tache noire, which is the typical eschar at the site of the tick bite. Immunoglobulin M (IgM) and IgG enzymelinked immunosorbent assay and the gold standard micro-immunofluorescent assay, allow serological diagnosis. We report the case of a man suffering from MSF, whose atypical presentation and false-negative diagnostic tests delayed consistently diagnosis and therapy. Afterwards we review the literature about this topic. Contributions: PM and AS studied the case, reviewed literature and wrote the paper; AB, TC and GP studied the case; AD'A and CC studied the case and reviewed literature; AC coordinated the study group, reviewed literature and wrote the paper.Conflict of interest: the authors declare no potential conflict of interest.