An 11-year-old girl presented with acute pancreatitis and mass in the head of the pancreas. MRI revealed a heterogeneous right-upper quadrant retroperitoneal mass measuring 6.8×6.1×5.5 cm arising from the pancreatic head. Endoscopic ultrasound with fine-needle aspirate revealed a solid pseudopapillary tumour (SPT) of the pancreas. The patient underwent a pylorus-preserving Whipple procedure. Pathology confirmed SPT. First described by Frantz, SPT represents less than 3% of all exocrine tumours. It is especially rare in children and shows different clinical features compared with adults. In our patient, tumour cells were arranged at the periphery of fibrovascular cores, but they did not show definite gland formation, keratinisation or cytoplasmic pigment accumulation. A periodic acid-Schiff stain without diastase did not show appreciable glycogen within the tumour cells, classic for Frantz's tumour. The literature, diagnosis, management and pathogenesis on this rare entity in children are reviewed and discussed.