Urinary tract involvement in EEC syndrome

A patient with fully expressed, sporadic ectrodactyly, ectodermal dysplasia, and cleft lip and/or palate (EEC) syndrome with renal involvement is presented; descriptions of four similar patients were found in the literature. Based on these patients, we suggest that the renal involvement is a form of dysplasia secondary to fetal urinary tract obstruction. We emphasize the importance of searching for a urinary tract anomaly in patients with this uncommon syndrome.