Introduction:

Solid-cystic pseudopapillary tumor or Frantz-Gruber tumor is a rare pancreas neoplasm, with a higher presentation in females in the first decades of life, with nonspecific symptoms and definite histopathological characteristics.

Objective:

To report a rare case of a solid pseudopapillary cystic tumor located in the head of the pancreas in a young patient.

Case presentation:

Presentation of a clinical case of a patient diagnosed with a Frantz-Gruber tumor in an uncommon site of the pancreas; besides the review of its epidemiology, clinical and histopathological presentation, treatment and prognosis.

Conclusion:

It is a rare neoplasm that occur in less than 1%, with benign behavior and with low probabilities of malignancy if its resection is complete.