Kaposi sarcoma is a neoplastic vascular disorder. It usually present on the skin of the upper and lower extremities, rarely in the mucosa of the head and neck. The most common sites reported are within the oral cavity, particularly on the palate. Other mucosal sites are rare. We present an unusual case where the primary manifestation of the Kaposi’s sarcoma was in the nasal mucosa.

A 56-year-old female of Mediterranean descent presented with a 1 year history of swelling on the left side of her nose, nasal obstruction and occasional minor epistaxes. Physical examination showed a firm and bulging polypoid mass which filled the left nasal cavity without cutaneous lesions.

Computed tomography (CT) demonstrated a tumor, measuring 77 mm in diameter, occupying the left nasal cavity causing erosion of nasal septum and extending posteriorly to the left choana and nasopharynx. There was bilateral cervical lymphadenopathy. Patient treated with chemotherapy alone. She was in a complete response after the first cycle. The patient received no further treatment. She needs a regular medical checkups that include a review of a patient’s medical history and a complete physical exam. She is in excellent local control over 12 months.

A review of the literature revealed that only seven cases of primary Kaposi sarcoma of the nasal cavity have previously been published and only two of them presented in a patient not associated with the acquired immunodeficiency syndrome. Here, we report the third case where the primary manifestation of the Kaposi sarcoma was in the nasal cavity in a patient with an adequate immune system.