Acute orchitis or a mass in testis usually evokes a neoplasm, a torsion or infectious disease for the clinician. Rarely, a systemic disease is involved. Nevertheless, testicular involvement occurs during vasculitis, Behçet or granulomatous diseases, but is uncommonly the first manifestation. Histologic changes after biopsy or orchiectomy usually give the diagnosis. Testicular localization of systemic disease does not change the general treatment.