Two cases of basal cell nevus syndrome

Two cases of Basal Cell Nevus Syndrome (BCNS) are reported. They were diagnosed as BCNS because multiple jaw cysts, skeletal anomalies, and many other symptoms accompanied although basal cell nevus was lacked. Cleft lip or cleft palate (bifid uvula) was associated with each case.Accordingly the ratio of association of cleft lip and/or palate is 15.6%, and this is far more than the ratio in the normal population. This fact supports the conclusion by Gorlin and Sedano that association of orofacial clefting does not appear to be due to chance.