Caudal appendage is a rare malformation which has since ever been interesting. We present the case of a girl in which a caudal appendage was discovered before birth. A throughout checking found an extension from this abnormality to the cordal spine. The baby is operated when 2months old. Differential diagnosis are spina bifida, teratoma and pilonidal sinus. Caudal appendage is one of the typical cutaneous finding with underlying spinal dysraphism, such as hair tuft or pigmented macule. The risk is a tethered cord syndrome that can lead to severe complication. The treatment is either surgery or expectation.