Lymphangioma are rare benign lesions of the lymphatic vessels, found mainly in the head and neck. Almost all cases of macroglossia in young children are caused by lymphangioma of the tongue. Aesthetic, occlusal, functional and psychosocial problems may arise as a result of this condition. The preferred treatment is surgery but in some cases sclerosant therapy may be used as an adjunct treatment. We report a case of lymphangioma of the tongue with macroglossia that was treated successfully with a Vshaped anterior glossectomy and discuss the surgical considerations.