Duane's retraction syndrome is sometimes associated with developmental ocular and extraocular abnormalities. This disorder may also be present in association with specific malformative syndromes. The authors report the clinical features of a seven-year-old female affected by Duane's retraction syndrome (D.R.S.), rectoperineal fistula, imperforate anus and other congenital malformations. From two extensive reviews of 106 reports published before 1982 and from a search (using Medline 1981-1994) for reports of congenital malformations associated with this syndrome, no cases of sporadic D.R.S. associated with the perineal malformations mentioned were found, even though imperforate anus is considered the commoner of hindgut malformation.