Abstract
Complete agenesis of the dorsal pancreas (ADP) is an exceedingly rare congenital anomaly, compatible with life. The first case was reported in 1911 and so far around 100 cases have been reported in the world literature. Majority of the patients with this anomaly are asymptomatic or associated with abdominal pain, hyperglycemia, diabetes mellitus, and acute or chronic pancreatitis. We present a case report of a 34-year-old male with ADP, diagnosed incidentally during radiological evaluation for abdominal pain. Magnetic resonance cholangiopancreatography confirmed the absence of neck, body, and tail of the pancreas along with duct of Santorini and the minor duodenal papilla. Because of its rarity of occurrence, clinical awareness of the ADP can expand the differential diagnosis and improve patient management in pertinent light of the world literature.