Tumors that arise in the retrorectal (presacral) space are uncommon lesions. Their diagnosis and management remain difficult. These tumors present with nonspecific signs and symptoms, thereby leading to a difficulty in diagnoses. For complete evaluation of the lesion, cross-sectional imaging, most importantly MRI, is required for diagnostic purposes and can distinguish between benign and malignant lesions, as well as the extent of resection. MRI is also utilized to determine the most appropriate surgical approach. Surgical removal leads to favorable outcomes for patients with benign purely cystic retrorectal tumors. Preoperative tissue diagnosis with transperineal and transsacral biopsies of solid or heterogeneous cystic lesions plays a role in determining the necessity of neoadjuvant therapy, which may decrease local recurrence after surgery and avoid an unnecessary delay in systemic therapy. However, to perform a biopsy, one must abide by certain principles during the biopsy.