Introduction: Primary adenocarcinoma of the bladder is a very rare disease that is difficult to treat. In this paper, we report the second case in the literature with primary mucinous adenocarcinoma of the bladder which showed complete response to FOLFOX4 (fluorouracil, leucovorin, oxaliplatin) chemotherapy regimen. Case Report: A 41-year-old man was admitted to our hospital with a diagnosis of primary adenocarcinoma of the bladder. Due to the similarity in histology with colon carcinoma, a FOLFOX4 regimen was started. Complete response was achieved at the end of this treatment. Today the patient is free of local or systemic disease. Conclusion: FOLFOX4 regimen may be a treatment option for primary adenocarcinoma of the bladder.

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