Glomus tumor is rare tumor which arises from glomus body and is most frequently found in the soft tissue of the extremities. The lung is a rare ectopic site, and a malignant glomus tumor arising from pulmonary parenchyma is particularly uncommon. To deepen our understanding on their imaging features, we report a case of malignant glomus tumor of pulmonary parenchyma confirmed with surgical histopathology and immunochemistry and review the medical literature on pulmonary parenchymal glomus tumors with emphasis on their imaging features.