Background: Rectal duplication is very rare congenital malformation encompassing 5% of all gastrointestinal duplications. Co-existence of other anomaly with rectal duplication is very rare too.
Case Report: A full term female child presented with an abdominal cystic mass on first day of life. She had polydactyly and in the abdominal sonography showed bilateral ureteropelvic junction obstruction with moderate to severe hydronephrosis. Other finding was leukocoria due to congenital cataract. Complete excision of mass carried out and histopathology confi rmed the mass to be a recral duplication.
Conclusions: The co-existence of this rare anomaly with polydactyly, ureteropelvic junction obstruction and congenital cataract is interesting and can fulfi lled the criteria of an unknown syndrom or sequence.

Keywords: Cysts, Polydactyly, rectal duplication, ureteropelvic junction obstruction, Congenital Cataract