Ano-genital granulomatosis encompasses the previously recognized clinical entities of vulvitis granulomatosa, posthitis granulomatosa, and ano-perineitis granulomatosa. We report three patients with ano-genital granulomatosis. The pathological features of the disease are lymphoedema and the presence of non-caseating giant cell granulomas. These granulomas are histologically indistinguishable from those found in both Crohn's disease and sarcoidosis, therefore, patients with ano-genital granulomatosis with accompanying gastro-intestinal or pulmonary symptoms should be investigated for the presence of Crohn's disease or sarcoidosis, respectively. The value of ano-genital granulomatosis as a unifying clinicopathologic concept is to provide a label for the affliction as well as to stimulate a careful search for possibly underlying systemic disorders, thus also permitting a more specific approach to therapy.