PEComas are a family of mesenchymal neoplasms that have in common the presence of a unique cell type, the perivascular epithelioid cell (PEC). PECs uniquely exhibit a distinct immunophenotype with expression of both melanocytic, particularly HMB-45, and myogenic markers. Nasal PEComas are exceedingly rare. To date, 14 cases have been described in the literature and with the exception of 6 cases, the rest consistently lack epithelioid cells and HMB-45 expression and are best classified as nasal hamartomas or angioleiomyomas with an adipocytic component. Nasal PEComas may closely resemble malignant melanomas since both entities share many morphologic, immunohistochemical, ultrastructural and clinical features. The distinction is of paramount importance as melanomas tend to display an aggressive behaviour with associated poor outcome. Herein, we report a case of nasal PEComa in a 19 year girl, focusing on the importance of light microscopic, immunohistochemical and ultrastructural features in accurately establishing the diagnosis.