Introduction: Complete agenesis of body and tail of pancreas is a very rare type of developmental anomaly of pancreas. It is important regarding its presentations of diabetes mellitus, pancreatitis, and exocrine insufficiency. Case Report: An old man had presented with atypical symptoms of obstructive jaundice with exocrine insufficiency. CECT helped to reveal the complete absence of the body and tail of pancreas with radiologically normal head with no signs of pancreatitis or mass lesion. Conclusions: The cause of agenesis of the dorsal pancreas is currently not well understood. It can also present lately as the presenting case. The presentations are usually related to secretory malfunctions. CECT is an initial investigation for diagnosis.