A tailgut cyst is a rare developmental lesion and usually is located in the retrorectal or presacral space. Extrahepatic hydatid disease has been reported in several locations including the pelvis and it often poses a diagnostic challenge. There are very few reported cases of primary perineal hydatid cysts. We present the multimodality imaging findings of a tailgut cyst and concurrent perineal hydatid disease in a 32-year-old male patient.