Background: Neoplasms showing perivascular epithelioid cell differentiation (PEComas) are uncommon tumors of the sinonasal tract. They are often misdiagnosed as angiomyolipoma or a simple benign tumor or polyp. We present a further case of a sinonasal PEComa and review the literature in an attempt to ascertain their malignant potential. Methods: Published evidence on invasiveness and characteristics were defined on systematic review. MEDLINE and EMBASE were searched from 1966 and 1980, respectively, to week 3 of December 2010. Publications reporting PEComa or angiomyolipoma were sought. Only those describing a sinonasal origin were included. Demographics, anatomic site, local invasion, recurrence rates, and mortality were recorded. A case report of a locally invasive intranasal PEComa is described. Results: In addition to the case we present, 12 case reports were located (n = 13). The mean age of patients was 59.6 (SD, 14.98 years) years. The location was within the right sinonasal tract in 54% of cases, the left sinonasal tract in 38% of cases, and not reported in 8% of cases. Treatment focused on local surgical excision and this was achieved endoscopically in 100% of cases. Recurrence rate was 8%. Mean follow-up was 17.4 (SD, 20.68 months) months. Invasion was noted in 23% of cases. There was a single death recorded. Conclusion: PEComas of the paranasal sinuses and skull base appear to have a biological behavior different from simple benign angiomyolipomas reported elsewhere in the body. PEComa may be more intermediate or malignant in clinical behavior.