Background: Kaposi Sarcoma (KS) is a rare neoplastic vascular disorder. The pathogenesis of KS remains controversial. However, suppression of the immune response was almost necessary. Classical KS most likely affects the male genitalia. Penile KS is the most common, while isolated scrotal KS has been unusual to see. In this case report, we review a rare case of scrotal KS in a non-HIV patient with history, clinical, laboratory, imaging findings. Presentation of Case: A 60 years old male patient, known to have ischemic heart disease. Presented with progressive painful scrotum swelling for 3 weeks. He had dysuria and gave a history of weight loss. Not complaining of prostate disease. By examination, there were swelling, warmness, tenderness to palpation in his scrotum. A biopsy was taken which was shown a Kaposi sarcoma. Discussion: Despite it names as a sarcoma, it is not considered a real sarcoma because the tumour does not arise from mesenchymal tissue. It arises from endothelium tissue. The pathogenesis of KS still not clearly understood and remains controversial. However, what we know is all forms of KS are strongly associated with HHV-8 infections. The partial or complete suppression of the immune response was almost necessary. Therefore, KS is usually associated with HIV infection. KS of the scrotum has been reported in non-HIV patients. Very few cases have been presented with scrotal KS. First reported case of an isolated scrotal KS around the world was in 1976. While In 1979 had been the first time to performed total scrotectomy and bilateral orchiectomy for scrotal KS patient. A Turkish study In 2011 reviewed only one patient had scrotal KS out of 32 cases with HIV-negative KS in the Middle East. In this case, the patient received 3 cycles of chemotherapy with no improvement so we preferred local excision because these lesions were small and stable for about 3 years. After 3 years of follow-up no recurrence was observed. Conclusion: Classical KS is generally observed in the lower extremities, it can rarely affects scrotal skin as isolated lesions. Thus. Genital examination should also include for those patients. In addition, the isolated scrotal Kaposi sarcoma is absolutely cannot be cured by medication. Adjuvant chemotherapy still in many cases show incomplete improvement. Orchiectomy might be the conclusive curative option.