More than 30 years ago, G. Curranino described a rare congenital malformations association, as three main clinical features, all sharing a common embryological origin: typical scimitar sacral malformation, hindgut anomaly, and presacral tumor. To date, more than 300 Currarino syndrome cases have been reported in literature. They clearly show that Currarino syndrome often displays a broad inter- and intra-familial phenotypic variability, leading to diagnosis divergences or late discoveries, when severe complications have already occurred. In 1998, heterozygous mutations of the HLXB9 gene–which is now the MNX1 gene since 2008 - located at the 7q36 locus, were identified as disease-causing in half of cases, with an autosomal dominant mode of inheritance. The MNX1 gene encodes the HB9 homeobox transcription factor. Considering the reported series and the phenotypic analysis, Currarino syndrome appears actually as a wide clinical spectrum due to a malformative sequence, which has started early in embryological development. It has a wide variability of expressivity, and could be revealed by four major clinical signs with terminal spinal cord anomalies in 70% of cases. Therefore, the syndrome should always be considered even when only a partial phenotype is present. The major complication is infection of the pre sacral mass, linked to the chronic intractable constipation, with a risk of diffusion to cerebro-spinal fluid. A second complication is the malignant degenerescence of the pre sacral teratoma, which is rare, but not exceptionnal.