Abstract

Familial Mediterranean Fever (FMF), which is characterized by acute attacks of fever and serosal inflammation, is a hereditary autoinflammatory disease. 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 fever attacks. Nine-year-old male patient was referred to the hematology department for splenomegaly and lymphadenopathy. In tests, hypergammaglobulinemia was detected. For the last two years, he had been suffering from abdominal pain for about two hours a day. Orchitis and arthralgia attacks were developed during monitoring. Upon identifying a homozygous M694V mutation in MEFV gene, he was diagnosed as familial Mediterranean fever. This case is presented to draw attention to rare manifestations of familial Mediterranean fever.