Presacral masses are unusual growths that have a limited differential diagnosis, typically not including neuroendocrine tumors (NETs). Classically, NETs are well-differentiated gastroenteropancreatic tumors of probable benign behavior. These tumors are associated with a typical morphologic pattern and involve the distal colon, rectum, and genitourinary tract; they are considered less aggressive, frequently asymptomatic, and rarely cause carcinoid syndrome, even when metastatic. Neuroendocrine tumors of the presacral region are extremely rare and few have been described in the literature. They have not been previously reported as being associated with imperforate anus.