Diffuse pulmonary lymphangiomatosis is a rare pulmonary disorder affecting the lymphatic channels from the mediastinum to the pleura. The disease usually occurs in children and young adults and frequently ends with death due to progressive course. Imaging findings of the disease are based on lymphatic involvement which appear as mediastinal soft tissue infiltration and thickening of pulmonary peribroncovascular bundles and interlobular septae. In this report, spiral and high-resolution computed tomography, and ultrasonography findings of severe form of this rare disease are presented. Furthermore, some lymphatic disorders, which are called with similar name but different appearances on imaging, are discussed.