The retrorectal-presacral space is part of the pelvic retroperitoneum, located posterior to the mesorectal fascia and anterior to the sacrum. It can be affected by a variety of tumors that develop from embryonic remnants derived from the neuroectoderm, the posterior intestine, and the notochord.

According to the predominant histopathology, they are classified into four groups: congenital lesions (65%), neurogenic (15%), osseous (10%), and miscellaneous, fig[1] which are further subclassified as benign or malignant. 

Developmental cysts account for up to 60% of all congenital retrorectal-presacral masses, with the retrorectal cystic hamartoma being the most common asymptomatic lesion in adults.

Chordoma and teratocarcinoma are the most common malignant congenital lesions in adults and children, respectively.

Small lesions are generally asymptomatic and are incidentally detected, while larger lesions can cause symptoms due to compression of pelvic structures.

Cross-sectional imaging studies are crucial for diagnosing lesions, as they help identify their origin and location. Magnetic resonance imaging (MRI) is preferred for complex pathologies and soft tissues, while computed tomography (CT) is more effective in showing bone destruction, calcifications, and distant metastases.