Cheilitis granulomatosa (CG), which presents clinically as persistent lip swelling, is characterized histologically by noncaseating granulomatous inflammation of unknown origin. CG may also be part of the classic triad of the Melkersson-Rosenthal Syndrome (MRS) and alone is considered by some to be an oligosymptomatic form of MRS. We report a case of CG associated with migraine-like headaches in a 44-year-old woman. The clinical presentation, histologic findings, and subsequent treatment are described. A brief review of the literature also is provided.