Aim: To report a rare case of cheilitis granulomatosa (CG) with pica disorder presented as chronic persistent lip swelling with an unusual eating disorder. Background: Cheilitis granulomatosa or Miescher's cheilitis is a rare, persistent, or recurring nontender chronic painless swelling of one or both the lips due to granulomatous inflammation, which was first described by Miescher in 1945. It can present as a monosymptomatic form or as a part of other entities of Melkersson–Rosenthal syndrome (MRS). Case description: A 14-year-old female patient presented with persistent diffuse swelling of the upper lip for 1 year. She also had an unusual habit of eating clay, mud, and potentially harmful items like flakes of paint for 1.5 years. Clinically, chronic persistent lip swelling involving upper lip and gingival enlargements were present along with systemic manifestations. Patient counseling with combination therapy showed excellent response with no signs of relapse at 6 months of follow-up. Conclusion: Cheilitis granulomatosa is a unique rare inflammatory disorder with many possible contributory factors and exact etiology is poorly understood. Clinical significance: This case report draws attention to constant exposure to an allergen that could be one of the possible etiological factors to CG secondary to pica eating disorder. Though it is a rare disease, research on the pathogenesis of CG and clinical trials evaluating the efficacy of the various treatments are needed to enhance our understanding of this disease, to improve the quality of life, as patients are very disturbed by the unsightly and distressing nature of macrocheilitis.