The diagnosis of the tailgut duplication cyst was based on the classic features of fluid-filled cyst found in the retrorectal/ presacral space with internal septation which is characteristic. It can be asymptomatic or present with perirectal symptoms.

Differential diagnosis includes epidermoid cyst, dermoid cyst, rectal duplication cyst, anal gland cyst, cystic lymphangioma, and anterior meningocele. The distinction of tailgut cyst from other presacral cysts is important because of the malignant potential of the tailgut cyst.