Lobster‐claw deformity of the extremities, clefting of the primary and secondary palate, ectodermal dysplasia, and atresia of the lacrimal system are common features of the ectrodactyly‐ectodermal dysplasia‐clefting syndrome (EEC‐syndrome). The patients often suffer from repeated infections of eyes, upper respiratory tract and urogenital system. To exclude an immunodeficiency as cause of the infectious predisposition in patients with EEC‐syndrome, we screened the immunosystem in four related patients with EEC‐syndrome. All patients were found to present normal immunoglobulin production, complement activity, lymphocyte‐, and granulocyte function. We conclude that recurrent infections observed in the EEC‐syndrome are not caused by an immunological defect, but seem to result solely from anatomical anomalies.