Management of anorectal malformations, especially that of proximal and more severe forms have developed significantly in the last 25 years. Echography and magnetic resonance imaging have improved the diagnostic accuracy, especially in terms of associated anomalies that are the main cause of fatalities among patients with anorectal malformations. Animal models, novel molecular biological techniques and knock-out gene models have widened our knowledge on aetiology and pathogenesis of these anomalies. From early 1980s, the posterior sagittal anorectoplasty originally designed by deVries and Pena has systematized the surgical management of anorectal anomalies leading to improved functional outcomes. Surgical mini-invasive techniques are increasingly applied to the management of these anomalies, although their role is as yet not clearly established. Long-term functional follow data are also available today indicating a reasonable favourable faecal and urinary control in the majority of patients with anorectal malformations. For the patients with less favourable functional outcome there are bowel and bladder management options that provide sufficient social continence.