Background and Aims Pseudo-Kaposi sarcoma, also known as acroangiodermatitis, is a rare disease in the field of vascular surgery. Hence, this article was conducted to investigate the clinicopathologic characteristics as well as the diagnosis and treatment of this disease through reporting a case of pseudo-Kaposi sarcoma caused by venous valve dysfunction, combined with literature review.Methods The clinical data of one patient with pseudo-Kaposi sarcoma were retrospectively analyzed, and the etiology, typology, clinical manifestations, pathological features, diagnosis, and treatment of pseudo-Kaposi sarcoma were summarized with domestic and foreign literature review.Results The patient was a 37-year-old man who had been engaged in physical work in a standing position for a long time. He had several dark purplish-red ecchymoses on the interior sides of the lower portions of both ankles and had pain after prolonged standing during the past month. He was diagnosed with "pseudo-Kaposi sarcoma" by combining clinical and pathological examinations and adjuvant examinations. After wearing elastic stockings, promoting venous reflux, anticoagulation, and improving circulation, there was no obvious pain in the skin lesion on both lower limbs, and no development or growth of the skin lesions were noted.Conclusion Venous valve dysfunction is associated with the occurrence and development of pseudo-Kaposi sarcoma, which is rare and difficult to make a differential diagnosis, and needs to be considered comprehensively according to the lesion characteristics, pathology, and angiographic features. There is currently no specific and effective treatment.