A recent review of the literature identified more than 150 reported cases of pancreatoblastoma in children. However, the clinical, histological and therapeutic characteristics of this tumour are hardly known by most paediatric surgeons and oncologists. The clinical symptomatology is often discrete, such as abdominal pain and/or intestinal transit disturbances, and the revealing sign is usually the discovery of a voluminous abdominal mass. Pancreatoblastoma is most often located in the head or body of the pancreas but can be seen in any part of the pancreas. It forms a full mass, rather well encapsulated, round and soft in consistency, often large in size and that can develop beyond the limits of the pancreatic gland. The metastases may be present in the lymph nodes, liver, lungs and spleen. It is an embryonic organ tumour that morphologically resembles what the nephroblastoma or the hepatoblastoma are for the kidneys or liver, respectively. The pathological analysis characteristically shows two components in which cell density is often high: an epithelial component and a mesenchymatic component. The lab test evaluation should include an assay of alpha-foetoprotein. Elevated levels of this marker are often present in these tumours. An assay of this marker is therefore interesting, not only at the time of diagnosis, but especially for early diagnosis of relapses. The pancreatoblastoma treatment is above all surgical and only complete exeresis makes recovery possible. However, at the time of diagnosis, many patients are inoperable due to the extension of the tumour. The combination of cisplatin + adriamycin seems to be the most effective neoadjuvant chemotherapy regimen. Patients who have had an incomplete tumour exeresis pose a real problem due to the frequency of local relapses and/or metastases. Local irradiation is indicated in this case, as chemotherapy has not yet provided proven results in this context.