Making the correct diagnosis for a lingual ulceration can be difficult. We present the case of a 55-year-old man, alcoholic and smoker who presented with a single lingual ulceration. The initial diagnosis suggested a cell-squamed carcinoma but histological examination revealed a Wegener Granulomatosis. Wegener Granulomatosis in the oral cavity is found in 6-13% of cases but rarely primary sign of the disease. The authors review differential diagnosis that should be taken into consideration when presented with similar lesion.