Malignant musculoskeletal tumour may cause considerable burden to general health. The fast growth combined with the tumour characteristics and its invasion capability resulted in the poor prognosis of malignant musculoskeletal tumour. Malignant musculoskeletal tumour may cause significant disability by destroying normal tissue that plays important role in body kinematics. Thromboembolism, including deep vein thrombosis, pulmonary embolism, and other kinds of venous thromboembolism, is one of the most underestimated complications of musculoskeletal tumour. Normally, thrombosis ensues when pathologic factors overcame the body hemostatic regulatory capabilities, which will predispose the body to the formation of thrombus. Venous thromboembolism in musculoskeletal tumour may develop as a result of interaction between the tumour pathologic capabilities and its interaction with normal bodily functions. In this study, we reviewed the burden of musculoskeletal tumour and its complication on global health. Then, the review will focus on the pathologic and clinical aspect of thromboembolism in malignant musculoskeletal tumour, including pathophysiology, diagnosis, and treatment based on recent findings and literature.