Purpose of Review <p>Pancreatic desmoid-type fibromatosis (DTF) are rare, locally aggressive, non-metastatic tumours that can mimic other pancreatic neoplasms, often leading to diagnosis dilemmas. This review collates contemporary literature to clarify the typical clinical, radiological and histopathological features, rationalise management strategies, and summarise outcomes to inform improve current clinical practices.</p> Recent Findings <p>Following a PRISMA-guided search to 20 August 2024, 26 histology-confirmed pancreatic DTF cases were identified. The median age in reported cases was 45 years with equal sex distribution. Lesions arose most commonly in the pancreatic tail and often presented with non-specific gastrointestinal symptoms. Imaging was heterogeneous and frequently misdiagnosed pre-operatively as mucinous cystic neoplasms, GIST, or adenocarcinoma. Diagnosis was confirmed on histopathology with nuclear β-catenin immunoreactivity. Surgical intervention was the treatment for most, with distal pancreatectomy being frequently utilised. Peri-surgical and post-surgical complications were generally manageable, however there one post-operative death was reported, and one case exhibited local recurrence. No distant metastases were reported. Emerging international guidance now supports active surveillance for asymptomatic or stable disease, reserving intervention for progression or symptoms.</p> Summary <p>Pancreatic DTF should be considered in the differential of atypical pancreatic masses. Tissue diagnosis should be guided with β-catenin immunohistochemistry rather than with imaging, given non-specific findings. While historical management has favoured surgical intervention, a surveillance-first approach is increasingly appropriate in carefully selected, stable patients, with surgery reserved only for symptomatic disease. Long-term follow-up is recommended due to the risk of local recurrence and the tumour’s unpredictable behaviour.</p>