MURCS association is rare, first described by Duncan et al. in 1979, including Müllerian duct aplasia, renal aplasia and cervicothoracic somite dysplasia. Levitt and Pe~na described in 2007 a classification of syndromic anorectal malformation (ARM) that associates these two entities. The reported case is the first one described in neonatal period. We describe a case of a female newborn with suspected diagnosis of anorectal and renal malformations. Physical and radiologic investigation revealed agenesis of sacrum and coccyx, tethered cord, left multicystic renal dysplasia, absence of vaginal orifice and hymen, normally placed urethral orifice and abnormal anal opening at the vaginal introitus as a rectovestibular type fistula. Also, she had right uterine, tube and ovary agenesis with a normal 46, XX female karyotype. A left diversing colostomy was done in first day of life and four months later, was performed a posterior sagittal anorectoplasty (PSARP), with intra-operative identification of a duplication of the distal rectum (related with caudal regression syndrome type 2). There were no complications in postoperative period. A staged management strategy is a viable option avoiding further complications in an already poor prognosis situation. ; info:eu-repo/semantics/publishedVersion