The authors describe the case of a 2-month-old male brought to the emergency room in a preagonal state cause by extensive septic necrosis of an enormous lymphangioma located on the left lateral trunk. In spite of emergency treatment the child expired shortly after admission. Our observation of this case prompted a review of the literature on the treatment approaches currently used for these tumors. Emphasis is placed on the necessity for early treatment in order to avoid infectious complications that, if neglected, can lead to unmanageable and possibly fatal sepsis.