Pancreatic perivascular epithelioid cell tumours (PEComas) are extremely rare tumours. A total of 34 cases have been described to date. The WHO defined it in 2016 as a mesenchymal tumour composed of perivascular epithelioid cells (PEC). They are characterised by a focal association with the vessel wall and simultaneous expression of melanocytic and myogenic markers. PEComas can affect a wide variety of organs and their morphology can vary, making their diagnosis challenging.1 PECs were first described in 1943 by Apitz et al.2 but it was not until 1992 that Bonetti et al. first used the term perivascular epithelioid cells according to their immunoreactivity and pathological characteristics. Tumours derived from these cells were termed PEComas.3 Subsequently, angiomyolipomas (AML), clear cell sugar tumours of the lung (CCST), lymphangioleiomyomatosis (LAM) and other atypical visceral neoplasms that may involve intra-abdominal organs, bone and soft tissues were included in the PEComa family. Currently, with the exception of AML, CCST and LAM, all other PEComas are referred to as non-specific PEComas (PEComas-NOS).4