Angiomyolipomas are benign tumors that can occur in the kidneys. Though venous invasion is a common complication of renal cell carcinoma, it rarely occurs with angiomyolipoma given its benign nature. We aim by this article to conduct an extensive literature review documenting rare cases of angiomyolipomas with venous extension, particularly with inferior vena cava invasion. The literature review was conducted following PRISMA checklist, using PubMed searching for English and non-English articles up to January 2020, and Mesh terms: angiomyolipoma, inferior vena cava, vein. Only cases reporting patients with angiomyolipomas with venous extension were included. Data extraction of different patients' parameters was conducted by two independent researchers in a uniform manner to a detailed datasheet. The search ended up with 90 cases, after excluding 116 cases for multiple reasons. Venous invasion of angiomyolipoma is a rare phenomenon. Clinical presentation is similar to any other renal tumor. Imaging is primordial for diagnosis and classification. Surgery is indicated as soon as venous involvement is demonstrated. In most cases, the prognosis is favorable with low morbidity and mortality.