The first time in Scandinavia we present a case report of a pure primary low-grade neuroendocrine carcinoma (carcinoid tumor) of the prostate. Our patient is a 34-year-old male with a long history of symptomatic chronic prostatitis/prostatodynia. After developing severe obstructive uropathy, a transurethral resection was performed. An unexpected diagnosis of a low-grade neuroendocrine carcinoma was made. Subsequently, in radical prostatovesiculectomy, we noted metastases to both seminal vesicles and two inguinal lymph nodes. Follow up is ongoing.