Chelitis granulomatosis is a rare, persistent, painless, idiopathic chronic swelling of the lip. It is a manifestations of orofacial granulomatosis(OFG), which is characterized by non- necrotizing granulomatous inflammation of the oral and maxillofacial region. Clinically, chelitis granulomatosa represents labial enlargement, perioral and mucosal swelling, and oral ulcerations. OFG is an increasingly recognized entity. The exact etiology of not clear, and therefore the precise treatment and long-term prognosis remain uncertain. Chelitis granulomatousa has no predisposition to race, sex, or age, and the incidence is 0.08 % in the general population. Clinician should know about the history, clinical features, etiopathogenesis, and proper investigations to diagnose the disease so as to provide various means of treatment. We present a case report of a 45-year-old male patient with histopathologically confirmed chelitits granulomatosa, which is diagnosed and managed with a nonsurgical treatment approach. The present case highlights the importance of thorough investigations in the diagnosis of this lesion, as the findings mimic many other granulomatous conditions.