Abstract: Paediatric pancreatic tumours by virtue of their rarity pose a significant challenge for the management.1 Although pancreatoblastoma and acinar cell carcinoma are distinct tumour entities, there is considerable overlap of histopathological characteristics which makes the diagnosis difficult pathologically.2 In infancy and childhood pancreatoblastoma is the most common tumour.3 Acinar cell carcinoma usually presents in older children.4,5 We describe a four year old child with acinar cell carcinoma which is possibly only the fourth case with this condition at such a young age.