Abstract: In current study we evaluated clinical features of Behcet ’ s Disease (BD) in patients without oral aphthosis (NOA cases). Methods. In a cohort of BD, patients registered during a period of 36 years were collected. We determined clinical features of BD NOA cases and compared them with patients with oral aphthosis (OA cases). The comparison was performed by chi square and Fischer ’ s exact test. Results. Among 6821 BD patients, 175 patients (2.56%) were NOA cases. Male/Female ratio was less in NOA cases (p-value: 0.078). Mean age of disease onset was signifi cantly higher in NOA cases (p-value: 0.001). Among NOA cases, the fi rst manifestations comprised uveitis (70.3%), joint involvement (8.0%), retinal vasculitis (6.9%), and genital aphthosis (4.0%). During the course of disease, the prevalence of ocular lesions and positive pathergy test were signifi cantly higher in NOA cases. Conversely genital aphthosis (OR: 0.048), mucocutaneous (OR: 0.470), joint involvement (OR: 0.478), and positive family history for BD (OR:0.138) were signifi cantly less frequent in NOA cases. NOA cases fulfi lled diff erent criteria including International Criteria for BD (ICBD), Japan Revised, Iran, Dilsen, and Classifi cation Tree. Conclusions. These results addressed the distinct clinical features in NOA subset of BD Including more prevalent eye involvement and positive pathergy.