Relevance. Intestinal lymphangiectasia is a very rare condition that causes damage to the lymphatic capillaries of the small intestine. This condition is associated with the development of lymphopenia. The purpose of this study is to provide up-to-date information on the etiology, pathogenesis, diagnosis, and treatment of patients with intestinal lymphangiectasia. The main part. There are primary and secondary lymphangiectasia. Primary lymphangiectasia is congenital and is most likely genetically determined. It is characterized by morphological failure of the lymphatic capillary wall, which manifests itself in the absence of valves, endothelial defects, and weakness of the connective tissue framework. This leads to overstretching and increased permeability of the lymphatic vessels, even when the lymphatic pressure is normal. Secondary develops against the background of a disease that leads to obstruction of the lymphatic vessels, which leads to an increase in lymphatic pressure. The consequence of lymphopenia is the formation of the syndrome of exudative enteropathy. Loss of plasma proteins leads to hypoalbuminemia, a decrease in plasma oncotic pressure and hypoproteinemic edema. Loss of lymphocytes turns into lymphopenia and secondary immunodeficiency. Loss of fats and vitamins leads to steatorrhea, hypocholesterolemia, deficiency. Conclusion. The key step in the treatment of intestinal lymphangiectasia is diet and symptomatic therapy. In the case of secondary lymphangiectasia, the priority is to eliminate the underlying cause of lymphostasis.