ABSTRACT: BACKGROUND: Scrotal lymphedema is rare outside endemic filariasis regions in Africa and Asia. It is of variable origin in the western world. CASE PRESENTATION: We present a case of a 40-year-old European man with massive elephantiasis of the scrotum attributed to chronic inflammation of the lower urinary tract caused by urinary outlet obstruction and diabetes mellitus. The patient underwent subtotal scrotectomy saving penis, testes and spermatic cords and followed by scrotal reconstruction with adequate cosmetic and functional outcome. CONCLUSION: In this report we discuss a rare case of scrotal elephantiasis in an European patient, reflect on the etiology and the diagnostic and therapeutic approaches. Surgery can be successful even in giant scrotal elephantiasis.