Granular cell tumour (GCT) is not an uncommon tumour of neural origin usually located on subcutaneous tissues and oral cavity. In prostate gland is exceptional, with only one case reported on the indexed literature of the last three decades. We report a case of a 63-year-old man presented with urinary complaints, enlarged prostate and increased PSA levels. The patient subsequently underwent transrectal needle biopsy which revealed GCT. The clinicpathological dilemma originated after this diagnosis is discused and the most suitable follow-up is proposed.