Liang-Liang Dong,1 En-Guo Chen,1 Irfan S Sheikh,2 Zhi-Nong Jiang,3 Ai-Hua Huang,3 Ke-Jing Ying1 1Department of Pulmonary Medicine, 2Department of Internal Medicine, Sir Run Run Shaw Hospital, 3Department of Pathology, Sir Run Run Shaw Hospital, College of Medicine, Zhejiang University, People’s Republic of China Abstract: Glomus tumor is an exceedingly rare neoplasm that is derived from cells of the neuromyoarterial glomus or glomus body. It rarely occurs in the visceral organs where glomus body may be sparse or even absent, such as the stomach, intestines, mediastinum, and respiratory tract. It is unusual for a glomus tumor to demonstrate atypical or malignant histopathological characteristics. It is also rare for such a tumor to express clinically aggressive behavior. However, when metastasis does occur,this disease is often fatal. We herein report an interesting case of a middle-age woman admitted due to progressive cough and hemoptysis. A polypoid mass was found to occlude the left lingular lobar bronchus. Final histopathologic examination showed the presence of malignant glomus tumor, confirmed by immunoreactivity for smooth muscle actin and vimentin. Two months later, the patient developed abdominal distension and gastrointestinal bleeding. Further evaluation lead to the discovery of widespread metastatic disease to the gastrointestinal tract, spleen, and the left adrenal gland. We further entail a review of the literature on the clinicopathologic features and diagnosis of this uncommon tumor. Keywords: malignant glomus tumor, lung, metastasis, diagnosis, immunohistochemistry