A three-day-old Montafon calf was brought to the clinic and died in spite of intervention attempts. In the necropsy of this calf, abdominal cavity was fulled with caseous material and intestines were distended due to gas accumulation, had an anemic texture. The liver tissue was pale gray-white in color. The duodenum was not present where it should normally be between the jejunum and the pyloric region of the abomasum. Instead, there was a structure presenting hyperemic and adipose tissue about 25-30 cm, along with lymph nodules. At the end of the Pylorus and at the beginning of the jejunum, there were luminal structures near the thick, crumpled crater-like circle. The pancreas was not present where it anatomically should be found. In the chest cavity, the lungs were congestion and not fully collapsed. The heart muscle was pale. In the lights of necropsy and histopathological findings, case was diagnosed as congenital duodenal agenesis, according to the authors knowledge this is the first report in a calf in Turkey.