Vascular tumors arising in the kidney are distinctly uncommon occurrences and have not been well-characterized in the literature; most cases have been documented in single reports or small case series. These tumors often exhibit nonspecific clinical and radiologic features and may mimic malignant epithelial tumors such as renal cell carcinoma (RCC) on imaging studies, necessitating nephrectomy with pathologic examination for definitive diagnosis. In addition, since primary renal vascular tumors are rare and infrequently encountered in surgical pathology specimens, they are often not considered in the differential diagnosis and therefore may be systematically under-diagnosed. In this review, we discuss the clinical and morphologic features of benign, borderline, and malignant vascular neoplasms and related lesions arising in the kidney, with a focus on recent, clinically relevant advances in imaging, histopathologic diagnosis, and treatment.