Introduction Pure small cell prostate cancer (SCPC) cases are very rare. Acute zonal occult outer retinopathy (AZOOR) has been described as a non-neoplastic retinopathy. We report the first case of pure SCPC preceded by AZOOR in the literature. Case report A 59 year old gentleman presented with an obstructed infected urinary system. He had a diagnosis of AZOOR 6 months ago that was investigated with full body imaging without any suspicious findings. However, the most recent CT findings demonstrated extensive disease dissemination. The patient underwent rigid cystoscopy and resection that confirmed a diagnosis of pure SCPC. Discussion AZOOR is a clinical syndrome of photopsia and rapid zonal field loss. The exact aetiology remains unknown and its association with malignancy remains contentious. Paraneoplastic manifestations of unexplained visual loss in SCPC are rare with only 2 cases reported in the literature. There are no cases demonstrating an association between AZOOR and SCPC. Conclusion Pure SCPC is an aggressive malignancy with most cases presenting with extensive disease dissemination on diagnosis. Early detection has a role in improving prognosis but is challenging. Further research is required to establish a standard treatment protocol.