Multifocal lymphangioendotheliomatosis with thrombocytopenia is an extremely rare disease. This condition manifests as diffuse congenital vascular lesions in the skin and gastrointestinal tract leading to severe gastrointestinal bleeding and thrombocytopenia. Histopathologic and immunohistochemical studies of vascular lesions demonstrate a lymphatic endothelial cell origin. Treatment often is not satisfactory. We herein describe a 4-week-old infant with this uncommon clinicopathologic entity.