Lymphangiomas are benign tumors resulting from a congenital malformation of the lymphatic system. Relatively uncommon, lymphangiomas are usually diagnosed at birth and develop within the first years of life. When these tumors occur in the oral cavity, the tongue is the most frequently affected site. Lymphangioma of the tongue is a common cause of macroglossia in children, which may lead to a dry/cracked tongue with ulcerating secondary infections, difficulty in swallowing and mastication, speech disturbances, exclusive nasal breathing, airway obstruction, mandibular prognathism and other possible deformities of maxillofacial structures. This paper discusses the most relevant features, clinical manifestations, disease-related impairments and treatment options for lymphangioma of the tongue. It presents the case report of a five-year-old child diagnosed with this lesion, including a description of the patient's dental care management.