A report is presented on a newborn with congenital malformations of the respiratory and gastrointestinal tract associated with total laryngo-tracheo-oesophageal cleft (type III). The history, the clinical course with respiratory distress after birth, the intra vitam diagnostic procedures with x-ray and laryngoscopy and the autopsy are reported. Additionally a situs inversus totalis, an aplasia of the right diaphragm and a hypoplasia of the lung on the right side were found. To the best of our knowledge this type of association has not been previously reported.