Intraductal papillary mucinous neoplasms (IPMNs) account for approximately 35% of all cystic tumors in the pancreas and represent the largest subgroup. They are characterized by mucin production and intraductal papillary epithelium growth. IPMNs range from benign to malignant lesions. Biomarkers combined with 18F-Fluorodeoxyglucose-positron emission tomography (18FDG-PET) is the best diagnostic tool. The risk of malignant transformation for main-duct IPMNs is between 34-68% and for low-risk branch-duct (BD)-IPMNs it is 1.1%. Monitoring is crucial for determining the optimal time of surgical excision. Novel artificial intelligence combining clinical, tumor biomarkers, imaging and molecular genomics plays a determinant role in the evaluation of such lesions. The first diagnostic tool is multidetector helical computed tomography (MDHCT) or up-to-date magnetic resonance imaging (MRI). MRI detects malignancy by enhancing mural nodules ≥3 mm. Novel endosonographic interventional techniques have been added to the diagnostic armamentarium. Pancreatoscopy is feasible and effective but challenging for evaluating the diagnosis, invasiveness, and extent of IPMNs. Its findings may change the surgical approach. Pancreatic juice and duodenal fluid have been used recently for molecular biological analysis. The genes most frequently altered include Kirsten rat sarcoma viral proto-oncogene (KRAS), tumor protein p53 (TP53), cyclin-dependent kinase inhibitor 2A (CDKN2A), SMAD family member 4 (SMAD4), and guanine nucleotide-binding protein, alpha stimulating (GNAS). Despite the advances in diagnostic modalities, assessment of this premalignant lesion of pancreatic cancer, with its poor prognosis, is a challenging task. Pancreatectomy is the indicated approach for malignant or high-risk IPMNs with potent malignancy. Conservative management or enucleation for preserving the pancreas of low-risk BD-IPMNs is recommended, but long-term follow-up for recurrence is necessary. The management of IPMNs must be individualized based on preoperative high-risk stigmata and worrisome features.