DFSP is a locally aggressive mesenchymal tumor that rarely metastasizes, and has intermediate malignancy. Head and neck may be involved in roughly 10 to 15% of patients; Among head and neck cases, involvement of immediate periocular region occurs in as low as 3.5% of such instances. we presented a systematic literature review on this situation along with clinical and paraclinical features of a case.

An electronic search was conducted on major medical sources. Data of the cases in the literature in addition to our own case were extracted, summarized and statistically analyzed.

We provided a case of primary involvement of DFSP neoplasm in medial aspect of left orbit. A total of 15 other cases from 14 relevant studies were also reviewed. Review topics included epidemiology, clinical presentation, pathologic features, differential diagnosis, imaging, treatment and, prognosis.

In summary, pri-orbital DFSP is a rare neoplasm and could be initially originated from pri-orbital area or from distant metastases. Due to its tendency to recur and importance of orbit organ, early diagnosis and taking proper therapeutic strategy is so important in such patients. However, occurrence of tumor in pri-orbital area is rare, in patients with non-tender, nodular firm, mobile mass with discoloration, DFSP should be one of the differential diagnosis of physicians.