“…However, the exact incidence of GU anomalies in EEC syndrome should be determined in larger number of patients. Various GU anomalies have been reported, including renal dysplasia (Rollnick and Hoo 1988;Moerman and Fryns, 1996;Frick et al, 1997), unilateral or bilateral renal agenesis (Rosselli and Gulienetti, 1961;Maisels, 1970;Kaiser-Kupfer, 1973), duplication of kidney (Preus and Fraser, 1973), duplication of urinary collecting system (Aldenhoff et al, 1978;Nardi et al, 1992), ureteric occlusion (Maisels, 1970;Frick et al, 1997), ureterocoele (London et al, 1985;Rollnick and Hoo, 1988;Moerman and Fryns, 1996;Frick et al, 1997), bladder neck contracture (Brill, 1972), unilateral hydronephrosis (Maisels, 1970;London et al, 1985;Rollnick and Hoo, 1988), bilateral hydronephrosis (Walker and Clodius, 1963;Johnson, 1976) and hypospadia (Rodini and Richieri-Costa, 1990). Although there were some reported cases of EEC syndrome with renal dysplasia, such cases with anomalies identical to the right nephrogenic cyst as a part of segmental dysplasia enclosing the main kidney had not been reported before.…”