Cutaneous cysts lined by ciliated epithelium are very rare, benign tumors predominantly occurring on the lower extremities of young women. We describe a ciliated cyst found in the perineum of a 60-year-old man. The location and histological features led us to attribute a primitive tailgut origin to this cyst, probably from the embryonic remnants of cloacal membrane. Only two previous examples of similar cysts were found in the literature.