Adenocarcinoma of the paraurethral gland is a rare tumour with no published prevalence in male patients. This report presents a case of bulbourethral gland adenocarcinoma in a 25-year-old man without comorbidities. After resection of the proximal urethra, sphincter and prostate, Mitrofanoff-type appendicovesicostomy was performed. In this case, chemotherapy or radiation was not included in the treatment as the surgery was estimated to be radical. The patient achieved good surgical and quality of life results. This case shows that macroscopic haematuria should be examined even in young age groups without comorbidities.