Inferior vena cava leiomyosarcomas are rare tumors that account for less than 0.7% of all retroperitoneal leiomyosarcomas. They are more common in women and cause nonspecific chronic abdominal pain. In this report, we present the case of a 53-year-old female patient complaining of chronic nonspecific periumbilical abdominal pain with initial onset 8 months previously who was diagnosed with inferior vena cava leiomyosarcoma by computed tomography angiography. The patient was treated with complete resection of the tumor and reconstruction of the inferior vena cava with interposition of a Dacron prosthetic graft. The treatment considered the gold standard consists of complete surgical excision, because these tumors are resistant to chemotherapy and radiotherapy. The prognosis of these patients is closely related to early diagnosis. Therefore, it is very important that vascular and general surgeons know that this disease is a possible differential diagnosis of chronic abdominal pains.