Lymphatic malformations are low-flow vascular anomalies of the lymphatic system that occur in one out of 2-4,000 live births. They may be classified as macrocystic (diameter > 1 cm), microcystic (diameter < 1 cm) or mixed. Lymphatic malformations that are not prenatally diagnosed are typically diagnosed at birth or in early childhood. The clinical presentation can be quite variable, ranging from a focal area with minimal swelling to large involvement with compromise of adjacent structures. Up to 75% of lymphatic malformations are found in the cervicofacial region and their presence in the aerodigestive tract can lead to life-threatening airway obstruction. Lesions can grow slowly or rapidly and suddenly due to infection, trauma, bleeding or hormone level changes. The paper describes the case of a 9-month-old male infant with cervical macrocystic lymphangioma appeared during a febrile airway infection and misdiagnosed with bacterial lymphadenitis.