A remarkable amount of new information has been generated on peritoneal mesothelioma (PeM), ranging from nomenclature changes, including the removal of “malignant” when referring to this neoplasm and the use of the term “tumor” rather than “mesothelioma” to designate the neoplasm formerly known as “well-differentiated papillary mesothelioma”, to the acknowledgment that PeMs can be associated with tumor predisposition syndromes or germline mutations. Although the disease is still more frequently seen in caucasian males, PeM is not uncommon in women. In addition, it can represent a diagnostic challenge when it has an uncommon presentation (ie, paraneoplastic syndrome or incidental finding) or when it has confounding histologic features. Ancillary testing, including immunohistochemical stains, in situ hybridization for CDKN2A or NF2, and molecular studies, in selected cases, allows its correct diagnosis. The molecular landscape of PeM is still a work in progress; however, some findings, such as ALK gene rearrangements and EWSR1/FUS-ATF1 fusions, are specifically seen in PeM of young patients. The biological behavior of PeM is variable; however, cytoreductive surgery and hyperthermic intraperitoneal chemotherapy have markedly improved the survival of patients affected by this disease.