Mucinous aenocarcinoma is one of the least common variant of prostate cancer. The prognosis of this variant of prostate cancer remains controversial. We report the case of 67-year old men with severe lower urinary tract symptoms (LUTS), bladder outlet obstruction (BOO), and sense of urinary retention. The serum level of prostate specific antigen (PSA) was 10.4ng/mL. Prostate was large (56mL), hard, lobulated, with extraprostatic extension and infiltration of surrounding structures, unsuitable for radical operative treatment. The patient demonstrated poor response to complete androgen blockade (CAB) (bilateral orchiectomy, steroidal antiandrogen) after 6 months of treatment with minimal decline of PSA (7.1ng/mL) and progressive growth of the primary tumor (110mL). The patient is submitted to antiandrogen withdrawal consisted of simultaneous addition of ketoconazole with hydrocortison substitution, resulting in normalisation of PSA (<1.0ng/mL) and diminution of prostate volume (PV) (63 mL) after 2 months, followed by adjunctive radiotherapy. Ketoconazole in reduced doses was stopped 6 months after completion of radiotherapy with PSA level < 0.002ng/mL and PV 16mL. The patient is alive with no evidence of disease more than 9 years after diagnosis of primary tumor. This case demonstrated modest hormonal sensitivity of mucinous adenocarcinoma of the prostate, whereas ketoconazole combined with radiotherapy, resulted in long term survival.