Angiomyolipomas (AMLs) are hamartomatous lesions containing smooth muscle, vasculature and mature adipose tissue. AML occurs most frequently in the kidneys, with the liver being the second commonest site of involvement. Other localizations for AML are extremely rare and, to our knowledge, only six cases of AML of the nasal cavity have been reported to date. Our case involved a 52-year-old male who presented with a history of snoring and obstruction of the left nasal cavity. Physical examination revealed a 3-cm polypoid mass in the left nasal cavity, which was excised surgically. Histopathologic examination revealed that the lesion was composed of smooth muscle bundles, mature adipose tissue and blood vessels of different sizes. No epitheloid smooth muscle cells were observed and HMB-45 staining was negative. In conclusion, although nasal AML is a rare lesion, it should be considered in the differential diagnosis of polypoid nasal masses, especially when the mass occurs unilaterally.