Ectrodactyly, Ectodermal dysplasia and Cleft lip/palate (EEC) syndrome is a rare combination of multiple congenital anomalies. Although the anomalies are diverse, the underlying problem arise from early embryonic ectodermal tissue defects or insults. We report a case of a baby boy who was born at 33 weeks of gestation with EEC syndrome. He died five months later of aspiration pneumonia. The management requires a multidisciplinary approach and proper parental counseling which should include risk of recurrence in future pregnancies. Prenatal diagnosis is possible by antenatal ultrasound scan at 14-16 weeks of gestation.