Introduction: Pancreatic acinar cell carcinoma is a rare malignancy accounting for less than 1% of all pancreatic tumors. Given its rarity, diagnosis is often difficult and delayed. There are no current standard treatment protocols and surgical resection is the only curative measure. This cancer is primarily seen in older males. However, we report a case of acinar cell carcinoma in a young male from Nepal who presented with jaundice. Case Report: A 30-year-old Nepalese male with no significant past medical history who presented with jaundice, generalized pruritus, dark urine, and a 34-pound weight loss in 1.5 months. He presented with elevated serum liver enzymes. An abdominal ultrasound was performed, which showed fatty liver, distended gallbladder containing large amounts of sludge, and dilated common bile duct with sludge. A subsequent MRCP confirmed a dilated distal common bile duct along with a filling defect, 2.0 cm from ampulla of Vater with no other significant abnormalities. His initial ERCP showed a stricture in the common bile duct, a sphincterotomy was performed, and a stent was placed. Cytology was nondiagnostic and only showed atypical glandular cells. A CT did not show any other lesions or gross abnormalities. Subsequent endoscopic ultrasound (EUS) showed a well-demarcated mass in the lower third of the common bile duct with no significant pathology in the pancreas. Another ERCP was performed, which showed a dilated common bile duct with distal stricture that was restented and nondiagnostic biopsies. The patient’s laboratory values showed a slightly elevated CA 19-9, normal CEA, negative ANA, and normal IgG4, making autoimmune pancreatitis unlikely. The patient underwent a pylorus-sparing pancreaticoduodenectomy (partial Whipple resection), abdominal lymphadenectomy, and cholecystectomy. Pathology showed a 3-cm high-grade acinar cell carcinoma in the pancreatic head. The final staging of the tumor was T3N1M0 with 2 out of 5 resected abdominal lymph nodes being positive for metastasis. All resection margins were negative. Tumor cells were positive for Cam 5.2, AE1/AE3 and anti-chymotrypsin, making it consistent with acinar cell carcinoma in addition to positive PAS (periodic acid-Schiff-diastase) staining of cytoplasmic granules. The patient was started on a course of radiation with 5 fluorouracil, 3 months after surgery, followed by chemotherapy with Gemzar. After 1 month post-adjuvant radiation and chemotherapy, the patient’s CA 19-9 decreased. At 12 months’ follow-up, the patient is doing well and is on oral medications for brittle diabetes. The patient’s follow-up PET scan was negative.