Retrorectal tumors are rare, the differential diagnosis is extensive, and their discovery is notoriously difficult and late. A high index of suspicion is needed to identify these patients. Once a benign or malignant retrorectal lesion is discovered and histologically diagnosed, it should be treated, even if the patient is asymptomatic. CT and MRI imaging can help differentiate between benign and malignant, cystic and solid and accurately define extent of adjacent organ and bony involvement to guide operative planning. Completely cystic lesions, in general, do not require preoperative biopsy unless malignancy is suspected. All solid tumors and heterogenous cysts should be considered for biopsy to rule out malignancy, guide neoadjuvant therapy, and plan extent of resection. Biopsies should be done transperineally or parasacrally. An aggressive approach, by an experienced, multidisciplinary team, that can achieve a tumor-free, en bloc resection, avoid tumor violation, restore spinopelvic stability, and minimize intraoperative and postoperative complications, should decrease the risk of local recurrence and improve survival.