We report the case of an 11-year-old female with acute pancreatitis, and review the literature highlighting the presenting symptoms and signs, laboratory tests, and investigational tools that helped in the establishment of a correct diagnosis. First, the patient presented to a regional hospital reporting abdominal pain, vomiting and liquid stool. She was admitted with the diagnosis of acute surgical abdomen and underwent surgery. Upon admission in our department, laboratory findings showed high values for total lipids = 2600 mg/dL and triglycerides 1200 mg/dL. Abdominal ultrasound revealed a pancreas with a small enlargement of the head (19 mm), and with a heterogeneous structure of the parenchyma. Abdominal computed tomography showed small left pleural collection and a high-dimensioned pancreas, particularly at the head, with heterogeneous structure, and peripancreatic collections. The patient was treated by fasting, gastric decompression by nasogastric tube, and intravenous antibiotherapy followed by antialgic and antispasticity treatment. Time of the patient’s first feeding was after the 7th day of hospitalization. The patient was discharged in a very good condition after 22 days of hospitalization.