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Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease characterized by acute attacks of fever and serosal inflammation. In untreated or inadequately treated cases, FMF is likely to cause amyloidosis that may result in mortality and morbidity. Patients usually come with a complaint of abdominal pain accompanied by febrile attacks. Nine-year-old male patient was referred to the hematology department with the indication of splenomegaly and lymphadenopathy. In tests, hypergammaglobulinemia was detected. For the last two years, he had been suffering from abdominal pain for about one or two hours a day. Orchitis and arthralgia attacks developed during monitorization. Upon identifying a homozygous M694V mutation in MEFV gene, he was diagnosed as familial Mediterranean fever. This case is presented to draw attention to rarely seen clinical manifestations of familial Mediterranean fever.

Splenomegaly, cervical lymphadenopathy and hypergammaglobulinemia; A case of atypical Familial Mediterranean Fever