The diagnosis of familial Mediterranean fever (FMF) is based on clinical evidence, since there is no specific diagnostic test. Manifestations are recurrent attacks of fever accompanied by serositis, mainly involving the peritoneum, pleura and joints. Although pericardial inflammation has been considered rare, when echocardiography is used to detect it, an incidence of 27% has been reported. We describe a boy of 11 and a girl of 15 years who developed recurrent pericarditis despite treatment with steroids, nonsteroidal anti-inflammatory drugs and pericardiocentesis. A few months after the first episode, both patients were admitted with typical bouts of FMF. Continuous prophylaxis with colchicine was initiated, and there were no further attacks during 18 and 10 month follow-ups, respectively. We conclude that acute or recurrent pericarditis in children or young adults of Mediterranean origin may be due to FMF.