Abstract
Neurothekeoma (NTK) is a specific benign soft tissue tumor, typically involving the skin of the head and neck area as well as the upper part of the body in young age with female predominance. It has a typical lobular pattern of growth but often displays atypical features such as myxoid stroma or fascicular pattern, which makes the diagnosis more difficult and may necessitate the use of immunohistochemical staining to differentiate NTK from nerve sheath tumor. Ocular NTK in general is very rare with only 11 cases previously reported. We are presenting a case of recurrent mixed cellular/myxoid NTK involving the lateral canthal area of a 16-year-old-boy and we demonstrate the diagnostic challenge in such cases to attract the attention of ophthalmologists and pathologist to the rare occurrence of NTK in the ocular region.