It is a rare disease that can be associated with the tuberous sclerosis complex, is characterized as low-grade metastasizing neoplasm, which leads to insidious cystic changes in the lung parenchyma and also affects the lymph vessels and lymph nodes and leads to chylothorax.12 The VEGF-D measurement was used to rule out this diagnosis in this case, as the marker is present at high levels in most patients and it is considered reliable for the diagnosis and evaluation of therapeutic response in these cases.12,13. The gene discussed is VEGFD; the disease is tuberous sclerosis.