Acute Pancreatitis (AP) is the inflammation and auto-digestion of the pancreas and is usually associated with the

elevation of serum amylase and lipase levels. Here, we report a rare presentation of AP with normal pancreatic

enzymes and lupus vulgaris (LV) in a patient with cystic fibrosis (CF). The chief complaints included severe

abdominal pain in association with fever and vomiting. On examination, the abdomen was tense and tender.

There were bilateral coarse crepitations with expiratory wheeze more pronounced on the left lower chest. After

detailed clinical evaluation, a provisional diagnosis of acute pancreatitis, with pneumonitis and the oral thrush

was established. Routine laboratory investigations were performed to confirm the diagnosis. However, the

serum amylase and lipase came out within the normal reference range. So, computerized tomography (CT) scan

of the abdomen was performed which showed a diffusely swollen and edematous pancreas along with peripancreatic

stranding, and hence confirmed the diagnosis. Afterward, the patient was managed conservatively,

and he had an uneventful recovery. The purpose of reporting this case is to promote awareness among fellow

healthcare professionals about this rare manifestation of AP and to prevent any missed diagnosis and serious

complications. To conclude, cystic fibrosis may have an unusual presentation like AP without pancreatic

enzyme elevation which may create a diagnostic dilemma. Hence, in such cases, a strong clinical suspicion and

supportive radiological findings play a critical role in the establishment of the diagnosis.