Introduction: Granulomatous cheilitis (GC) is a rare idiopathic inflammatory disorder characterized by chronic persistent swelling of the lips. Despite a lingering controversy, it remains linked to orofacial granulomatosis and Melkersson–Rosenthal syndrome (MRS). Aim: To systematically review all cases of GC reported hereto from India with the three being reported now, analyzing patients'/disease characteristics, associated diseases, and treatment protocol. Materials and Methods: Using biomedical search engines such as PubMed, Scopus, and Google Scholar, a systemic review of cases/case series reported from India (1994–2018) was undertaken, and data on patients'/disease characteristics, association with other diseases, and treatment extracted discussed and analyzed without subjecting to statistical analysis due to the small population size. Results: The mean age at presentation of these cases (n, 73: Single, 39: Participants of 6 series, 31 plus presently reported, 3) was 29.36 years and at onset was 26.62 years. There was female preponderance of 1.8. Complete triad of MRS was recorded in 8 (10.95%), and oligosymptomatic forms were as follows: GC + facial palsy, 5 (6.85%); GC + fissured tongue, 4 (5.48%); and facial palsy + fissured tongue, 1 (1.37%). Monosymptomatic GC was seen in 55 (75.34%) and upper lip involvement in 29 (39.72%). Conditions associated with GC included Down syndrome, rosacea, granulomatous vulvitis, and pregnancy. None of the cases were associated with Crohn disease and sarcoidosis. Intralesional triamcinolone acetonide (33, 45.21%) was the most common modality of treatment. Conclusion: Cases of GC from India over the past quarter of a century were reported rarely and showed female preponderance. This chronically recurrent entity with variable severity lacks larger studies and randomized controlled trials for optimum treatment.