Primary hydatid cyst in the head of pancreas is rare. We report a case of a 26-year-old patient, who presented with obstructive jaundice. The imaging studies, while demonstrating a fusiform dilatation of the common bile duct, was unhelpful in identifying the etiology. A pancreatic head cyst was discovered at the time of surgery. Diagnosis was confirmed by subsequent serology and identification of scolices on microscopic examination. From the reported data, we discuss the diagnostic and therapeutic modalities of this rare disease.

Highlights Polysplenia is a complex polymalformative syndrome that includes a spectrum of visceral and vascular anatomical abnormalities; extremely undiscovered in adulthood. A rare case of polysplenia syndrome showing an exceptional association between preduodenal portal vein, dorsal pancreas agenesis and polysplenia on gastric adenocarcinoma. Surgeons must be aware of their possible existence and be able to recognize them to avoid major intraoperative injuries. Surgeons must have a perfect knowledge of the reference anatomy and the exploration of anatomical variations in imaging before surgical exploration. PDPV is believed to pose increased risk to gastric cancer patients during gastrectomy and lymph node dissection around the hepatoduodenal ligament.

The diagnosis should then be considered early, and chest radiography with a nasogastric tube is the first technique to prefer and may be helpful to confirm the diagnosis.

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