Mucocele is a common condition of the minor salivary glands that is generally found on the lower lip. Although etiology is uncertain, mucocele are usually caused by local trauma. Cases involving infants are rarely reported in the literature. The objective of this study is to report an unusual case of mucocele in the fi rst year of life that was treated with surgical removal and postoperative follow-up. This report illustrates the main features of clinical interest and discuss classifi cation, differential diagnosis and the treatment approaches described in the literature.