Aim and background Small cell carcinoma of the prostate (SCCP) is a relatively rare entity, an aggressive tumor with a tendency to metastasize early. There is no standard chemotherapy regimen for SCCP. We report the case of a 67-year-old man with an initial diagnosis of prostate adenocarcinoma with bone metastases who subsequently developed a small cell carcinoma of the prostate with liver metastases. Methods and study design A review of this case and of the current literature was done with a view to highlighting the ideal approach to such an unusual tumor. Results and conclusion The patient was treated with carboplatin and etoposide and after 3 cycles achieved a good response. Unfortunately he developed massive pulmonary embolism which impeded the continuation of treatment and caused his death. By reviewing the literature we found there is no standard of care and new targeted therapies have failed to prove any benefit so far. It is mandatory to persevere in planning trials and exploring new drugs to improve the outcome of patients with SC-C P. It might also be worth creating a register for SCCP where data can be collected to coordinate the management of these patients and to establish better connections among cancer centers.