Nasal angiomyolipomas are exceedingly rare and usually present as small tumors in middle-aged or old men. Their histology is similar to that of the renal counterpart, but they differ in the absence of epithelioid cells, the lack of HMB-45 immunoexpression and no association with tuberous sclerosis. A case of angiomyolipoma occurring in the right nasal cavity was presented in a 44-year-old male patient. The mass was removed by an endoscopic approach. A well-circumscribed, 1.3 cm-sized, ovoid mass was composed of yellowish gray, solid, soft tissue. Histologically, it consisted of bundles of smooth muscles, mature fat cells and thick-walled blood vessels. Nests of seromucous glands were admixed within the mass. Neither epithelioid nor pleomorphic cells were found, and HMB-45-positive cells were not detected. No recurrence has been detected for 2 months after the operation.