International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties.Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations. Orofacial granulomatosis (OFG) is a lesser known disease entity, which presents a confounding spectrum of oral manifestations. It is a non-specific granulomatous inflammation caused by an elusive etiopathogenesis. It usually manifests as persistent and/or recurrent upper and/or lower labial enlargement. The clinical features, both extraoral and intra-oral, are highly variable and at times, insidious. This disease can be both cosmetically and functionally debilitating. Case Report: This is a report of an unusual case of orofacial granulomatosis which presented as a persistent severe generalized gingival enlargement in a nine-year-old child. There was no evidence of an underlying allergic or systemic cause. Surgical intervention (i.e., gingivectomy) showed no alleviation of symptoms. Conclusion: Making a prompt and precise diagnosis of orofacial granulomatosis is often challenging, however complete remission is possible with targeted therapy.