Familial Mediterranean fever (IMF) is a recurrent self-limiting polyserositis. Polyarteritis nodosa (PAN) complicating IMF is very rare. Here, we present a 17-year-old male patient with IMF who subsequently developed PAN 2 weeks after hepatitis A infection. This case was also complicated with perirenal haematoma, and right nephrectomy was performed. The clinical condition of the patient was improved after therapy with intravenous and oral corticosteroid and intravenous cyclophosphamide. However, the IMF attacks and vasculitic skin lesions again occurred while he was using colchicine plus immunosuppressive agents a few months later. Interferon-alpha therapy was administered and the attacks were resolved within 3 months. He has not experienced any other symptom during the follow-up period of 28 months.