Solid-pseudopapillary tumour of the pancreas is a rare benign or low-grade malignant epithelial tumour; its association with pancreatic dorsal agenesis has been reported only once before. We present the radiological and histological findings of a case of pancreatic solid-pseudopapillary tumour associated with total pancreatic dorsal agenesis. A 49-year-old woman presented with abdominal pain radiating to the back for several months. Radiological findings showed absence of the dorsal pancreas and an 8 cm x 6 cm diameter tumour arising from the head of the pancreas. She underwent successful complete resection of the tumour. Histopathology revealed a diagnosis of solid-pseudopapillary tumour.