Lymphangiomas are benign tumors that occur predominantly in the head and neck regions. They are very common in children and are usually diagnosed at birth or during the first years of life. Understanding their clinical features is crucial to an early diagnosis. The purpose of this paper was to report the case of a 3-year-old patient with a nodular lesion that evolved for 2 years on the tongue's dorsal surface, highlight the surgical treatment and characteristics of this kind of pathology, and explore the differential diagnosis.