Kaposi sarcoma (KS) is one of the most common neoplasms diagnosed in HIV- seropositive subjects. KS is an angioproliferative disorder of endothelial origin and is a locally aggressive multicentric mucocutaneous malignant neoplasm. Aggressive HIV-KS is associated with increased HHV8 viral load, disseminated lesions, and intraoral exophytic lesions, and may also present with facial lymphoedema which may be life-threatening or mild. A 26 -year-old, male, presented with severe oral pain and facial swelling. He had multiple oral lesions and an incisional biopsy confirmed Kaposi sarcoma. A multiple site incisional biopsy and immunohistochemistry for HHV8 was done and based on the clinical, histopathological, and immunohistochemical results a diagnosis of HIV associated oral and cutaneous KS with severe lymphoedema was confirmed. This aim of this case presentation is to describe a case of immunodeficiency virus-related lymphadenitis, oral and cutaneous KS associated with the complication severe lymphoedema in a HIV infected patient who was not on HAART therapy for three years after being diagnosed with HIV.