Orofacial granulomatosis is a disorder of the orofacial soft tissues characterized clinically by recurrent to persistent lip and facial swelling and histopathologically by noncaseating granulomatous inflammation, lymphangiectasia, and perivascular lymphocytic inflammation.1,2 It is a diagnosis of exclusion considered to represent a spectrum of clinical entities including cheilitis granulomatosa and Melkersson-Rosenthal syndrome. We describe herein a case of granulomatous stomatitis presenting with atypical gingivitis consistent with orofacial granulomatosis. Since granulomatous stomatitis may be a marker for systemic disease, it is imperative to rule out other potentially associated conditions.