Pancreoblastoma is a very rare tumor that originates from the exocrine epithelial cells of the pancreas. However, it is the most frequent pancreatic tumor in children. It usually appears in the first decade of life with an average of 5 years old. The clinical manifestations are usually unspecific, being the abdominal pain and the abdominal mass the most frequent. The radiological signs are not very well described in medical literature. The definite diagnosis should always be established with biopsia and histological examination. Here it is presented a clinical case of pancreoblastoma with unusual involvement of a major duodenal papilla in a pediatric patient.