CASE PRESENTATION: A 60-year-old female patient presented to us complaining of cough for 3 months. Physical examination reveal no significant findings. CT revealed a 2.5x1.5cm broad based enhancement at lingual segment (1A, B). Bronchoscopy revealed a white polypoid tumor occupying left lingular bronchus (1E). Biopsy was sent for pathology which revealed inflammatory exudates. Two months later the patient presented again to us with worsening of her symptoms. Another bronchoscopy showed the same white polypoid tumor with protrusion into the left main bronchus (1F). The tumor was excised by high-frequency electric snare ligation and sent for pathological examination. An abdominal CTdemonstrated multiple mass-like lesions occupying the gastrointestinal cavity, spleen and left adrenal gland (1C, D). An upper endoscopy was performed with ligation of mass-lesions in the fundus, antrum and duodenum (1G, H). Pathology ofthe specimens revealed uniform polygonal cells with nuclear atypia, necrosis andmitotic activity. IHC of the biopsy specimens stained positive for SMA and vimentin (2), and was partially positive for CD99and F8. The staining patterns supported the diagnosis of MGT. Finally, the symptoms worsened with massive hemoptysis and gastrointestinal bleeding. The patient died of multiorgan failure.