We report histopathological findings in a case of familial Mediterranean fever (FMF) syndrome with an erysipelas-like erythema (ELE). ELE is the only pathognomic cutaneous manifestation of FMF. ELE is characterized by well-demarcated, tender, erythematous and infiltrated plaques recurring on the same site and resolving spontaneously within 48–72 h. FMF is a monogenic autoinflammatory syndrome highlighted by recurrent fever associated with polyserositis involving mainly the peritoneum, synovium and pleura. FMF results from a mutation of the MEFV gene, which encodes for pyrin, leading to Il-1β activation and promoting neutrophil migration into the dermis. Histopathological findings in our case showed a sparse superficial perivascular and interstitial lymphocytic infiltrate admixed with some neutrophils, no eosinophils and mild papillary dermal edema. Venules and lymphatics were dilated, though no vasculitis was identified. Neutrophils are the most common cutaneous marker of autoinflammation, and cutaneous manifestations of monogenic autoinflammatory syndromes are represented by the spectrum of aseptic neutrophilic dermatoses. Neutrophils in the presence of recurrent fever and in the correct clinical context of recurrent erysipelas in the same site are a diagnostic clue for FMF.