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As a result of our data analysis we were able to draw an overview of the rare differential diagnoses and diagnostic management of acute scrotal pain. Conclusion Rare differential diagnoses of acute scrotal pain are susceptible to misinterpretation as testicular torsion or acute epididymo-orchitis. Surgical management is indicated in case of suspicion for torsion or tumor. We herein present knowledge of the rare differential diagnoses and raise awareness for associated systemic disease in order to facilitate disease management and increa