Lymphangioleiomyomatosis (LAM) is a rare disease that affects women in fertile age and presents a systemic progressive evolution, being the lung and the mediastinic lymph nodes the most affected organs. The pulmonary disease is characterized by dyspnea, pleural effusion, hemoptysis and spontaneous pneumothorax, being the chylothorax a frequent complication in the course of this disease, appearing in up to 30% of cases. The treatment of chylothorax is not standardized and it is necessary a multidisciplinary approach: nutritional, pharmacological, respiratory and even surgery. These patients present high risk of malnutrition due to the constant loss of chyle, therefore a suitable nutritional management is essential to avoid more complications.