Introduction and Importance: Leiomyosarcomas of the inferior vena cava are exceedingly rare tumors, presenting diagnostic dilemmas due to their nonspecific clinical features. This case report aims to shed light on the challenges associated with diagnosing and managing such tumors, emphasizing the importance of a multidisciplinary approach. This article uses SCARE2023 criteria as a framework to sort out a case report in order[1]. Case Presentation: We present a case of leiomyosarcoma of the inferior vena cava in a patient presenting with abdominal pain. Initial clinical signs, supported by computed tomography (CT) and magnetic resonance imaging (MRI), led to the suspicion of the tumor. Histological analysis confirmed the diagnosis, prompting surgical intervention. Clinical Discussion: The diagnosis of tumors within the inferior vena cava is often challenging due to their nonspecific presentation. Imaging modalities such as CT and MRI play an important role in describing the extent of the tumor and guiding surgical planning. Histological analysis remains supreme for definitive diagnosis. Surgical resection, aimed at complete excision, is the keystone of treatment to minimize the risk of recurrence. Conclusion: Leiomyosarcomas of the inferior vena cava pose diagnostic and therapeutic challenges due to their rarity and nonspecific clinical presentation. A comprehensive approach integrating clinical assessment, imaging, and histological analysis is essential for accurate diagnosis and optimal management. Multidisciplinary collaboration is key for navigating the complexities associated with these rare vascular neoplasms, ensuring positive patient outcomes.