Background: Diffuse lymphangiomatosis is a rare disorder of the lymphatic vessels. Its prognosis is poor and its treatment not codified.
Methods: We describe the case of a 14 years old boy known for a lymphatic malformation of the left chest wall with pleuropulmonary, bone and visceral involvement. He presented with respiratory difficulties and a left pleural effusion. The treatment associated thoracic drainage, surgical pleurectomy and iterative sclerotherapy.
Results: The patient is currently asymptomatic. On imaging, there is a decrease of the left lateral thoracic component and a resolution of the intra-pleural portion.
Conclusions: The treatment of diffuse lymphangiomatosis is based on a multimodal management.