Abstract: primary leiomyosarcoma of the inferior vena cava (PIVCLMS) is a rare soft tissue sarcoma with insidient onset and atypical along with nonspecific imaging findings. The diagnosis of PIVCLMS is relatively difficult, the misdiagnosis rate is high, lacking standard diagnosis and treatment means and methods, and the prognosis of patients with this disease is poor. In this paper, based on the literature related to PIVCLMS at home and abroad in recent years, the relevant researches on PIVCLMS were reviewed in order to improve the clinical understanding of PIVCLMS, carry out early diagnosis and treatment, and improve the prognosis of patients.