Background: Urethral adenocarcinoma is a very uncommon problem in women, with a prevalence of 0.02%. Due to the disease's rarity, there is very little information available about it. Treatment for cancer can have a significant negative influence on a patient's condition. Mucinous adenocarcinomas are a very uncommon type of cancer that is poorly studied. These cancers almost invariably have a worse outcome compared to conventional adenocarcinomas. Case Presentation: A 67 year old woman presented with hematuria and stranguria. A computerised tomography (CT) scan of this patient revealed an urethral tumor. In her histopathologic report we found the tumor cells were arranged in small clusters and single cells were scattered among extracellular mucin and signet ring cell picture, which is consistent with mucionous adenocarcinoma. We then performed partial urethrectomy with negative surgical resection, but the tumor reappeared 18 months later with no symptoms. The patient then decided to undergo radiotherapy for 33 sessions. From a recent MRI follow up the patient remains recurrence free. Conclusions: Early surgical treatment with or without adjuvant radiotherapy appears to be the best option in cases of small, organ-confined disease of urethral mucinous adenocarcinoma. Partial urethrectomy can be performed in this type of cases, which can prevent the use of permanent urinary catheters and further improve the patient's quality of life.