Granulomatous cheilitis is a rare disorder characterized by intermittent swelling of one or both lips, which may become persistent. We report a case occurring in a child, which improved with minocycline treatment.An eight year-old girl was seen for the swelling of her upper lip, which persisted for more than one year. There was no history of applied irritants, local trauma or atopy. On examination, the upper lip was swollen, indurated, erythematous and fissured with a bilateral perleche. The tongue and gums were normal. There was no facial palsy. The girl was in good health and did not take any drugs. Blood investigations were normal. Patch tests were negative. Antibiotic treatment or local steroids provided only temporary improvement. A biopsy showed extravascular epithelioid and gigantocellular non-caseating granulomas with lymphoid infiltrates. There was no sign of sarcoidosis or Crohn's disease. Treatment with hydroxychloroquine for three months, then with oral metronidazole, did not improve the symptoms. Minocycline (100 mg/d) was effective.Granulomatous cheilitis should be considered in children as well and differentiated from infectious, allergic or factitious dermatoses. Granulomatous cheilitis can be associated with Crohn's disease or sarcoidosis, and could precede these disorders from several months or years, thus requiring a long-term follow-up.