Background & Aim: The most common form of Von Recklinghausen is Neurofibromatosis. Malignant peripheral nerve sheath tumors (MPNSTs) may develop in five percent of the patients. The aim of this study was a review on Persian literatures about clinical findings in patients with Neurofibromatosis type I (NF1).

Material & Methods: In this narrative literature review researchers searched keyword "Neurofibromatosis Type 1" in Persian Google Scholar, and National databases; Scientific Information Database (SID), and Magazines Information Database. A total of 50 articles were retrieved from 2004 to 2015. Only 6 articles (three descriptive and three case reports) had inclusion criteria and were evaluated.

Results: The review of the studies showed; NF1 severity varies widely from mild to severe and even life-threatening. NF1 is typically diagnosed clinically during childhood (around age 5-10 years). Some of complications of NF1 do not appear until adolescence period and develop over adulthood and may progress to malignancy. The complications that reported in NF1 patients were malignancy, severe skeletal complications, dissecting aneurysm of the Aorta, cutaneous neurofibromas, neurological complications develop from tumors, malformations, and paraplegia.

Conclusion: Patients with NF1 experience complications include; malignancy, musculoskeletal, vascular and neurological side effects that some of them are seriously life-threatening.