A tailgut cyst is a rare congenital lesion that develops from a residual posterior remnant of the intestine and presents as a mass in the presacral space. They are generally asymptomatic or have atypical symptoms, are usually benign but may rarely become malignant. We report a case of a 37-year-old female who initially presented to the Surgical De-partment of Korle Bu Teaching Hospital, Accra, Ghana with a malignant tailgut cyst after having repeated surgical procedures for recurrent perianal infective pathologies but still had persistence of symptoms. The lesion was initially excised and found to be a dermoid cyst histologically. The mass recurred after a year and had a re-excision; the lesion was diagnosed histologically as mucinous adenocarcinoma. This report emphasizes the different forms of presentation of a patient with a tailgut cyst and the possibility of malignant transformation, as well as the presentation of this tailgut cyst which can be diagnosed using radiological investigations and histological findings.