Abstract: Introduction: Different methods of treatment were employed in ocular lesions of Behcet’s disease (BD) in adults. Most of them are not permitted in children due to their potential toxicity. Methotrexate (MTX) has gained wide acceptance for the treatment of childhood rheumatic diseases. On the other hand, we showed in several reports the efficacy of MTX in the treatment of ocular lesions of BD. This study was designed to show the effect of MTX in juvenile BD. Materials & Methods: Eighteen patients diagnosed as BD (according to the Iran and to the Japan criteria) with posterior uveitis (PU) and/or retinal vasculitis (RV) were selected. The disease had developed before age 16 in all (ranging between 7 to 16 with a mean of 13.7 + 2.6). They all received MTX, 7.5 mg/week orally in 3 divided doses, in addition to Prednisolone (0.5 mg/Kg/day orally), A disease activity index (DAI) based on the inflammatory state of each section of each eye, visual acuity (VA), and a total adjusted DAI for each patient was calculated. Improvement and stabilization of the lesions were accepted as a good result. The comparison of these data before and after the treatment was made by the student paired t test. Results: The age of the patients at the disease onset ranged between 6 to 16 with a mean age of 11.4 + 2.5. In 16 patients the ocular involvement occurred before the age 16 and in 2 after that age (mean age = 13.2 + 4.5). The mean age of the patients when starting MTX was 17.4 + 4.3. Only 10 patients received the treatments before the age of 16. The mean follow up time was 14.3 months (3 to 65). We had a good result for anterior uveitis (AU) in 89% of the eyes; the mean DAI decreased from 2.5 to 1.2 (t = 2.604, p<0.02). In PU the good result was seen in 90% of the eyes; the mean DAI decreased from 2.3 to 1.1 (t = 4.325, p<0.0003). RV showed good result in 85% of the eyes; the mean DAI decreased from 1.8 to 0.8 (t = 2.315, p<0.04). VA improved in 81% of the eyes; the mean VA I increased from 3.3 on 10 to 4.5 on 10 by the Snellen chart (t= 2.298, p<0.03). The total adjusted DAI improved in 78% of the patients, and stabilized in 5%. The mean total adjusted DAI decreased from 36.4 to 24.2 (t = 3.352, p<0.004). Adverse effects included mild and reversible increase in liver enzymes seen only in 2 patients (11%). There was no need to discontinue the treatment. Conclusion: MTX is effective in ocular lesions of BD in children, notably in posterior uveitis. It is well tolerated with minimal side effects in long-term use. MTX may be the drug of choice in severe ocular lesions of BD in children.