The granulomatous inflammation seen in filariasis, orofacial granulomatosis (OFG), rosacea and sarcoidosis can be associated with lymphoedema. In the setting of OFG, the finding of intralymphatic granulomas has been reported as a possible mechanism for lymphoedema. Anogenital granulomatosis (AGG) is a similar chronic inflammatory condition of unknown pathogenesis. It presents as granulomatous genital or anoperineal inflammation and associated lymphoedema, with histological findings of non-caseating granulomas and a perivascular infiltrate. We report a case of AGG and lymphoedema with intralymphatic granulomas seen on biopsy. This finding is unique and we propose that the intralymphatic granulomatous inflammation causes a partial or complete occlusion of lymphatic drainage, thus resulting in the clinical situation of lymphoedema.