Behcet disease (BD) is a multisystemic autoinflammatory disorder characterized by recurrent mucocutaneous, ocular, musculoskeletal, gastrointestinal, central nervous system, and vascular manifestations. Pulmonary arterial involvement (PAI) of BD is probably the most severe form of vasculitis, at least in children. PAI has a high mortality, morbidity, and recurrence rate. There are limited data regarding treatment and outcomes of pediatric patients with BD with PAI. Herein, we report 2 pediatric patients with BD presented with hemoptysis and support our data with a systematic review. These patients were given immunosuppressive therapy, which covered pulse methylprednisolone followed by oral prednisolone, intravenous cyclophosphamide every 3 weeks for a total of 6 cycles, and interferon-alpha 2a concomitantly. These are the first reported cases in the literature successfully treated with this treatment modality in a complication with 50% mortality. These patients have been followed up for a period of at least 4 years without any vascular recurrence. Pediatricians should be aware that patients with BD may not present with full diagnostic criteria. They should consider BD in a child with PAI to avoid diagnostic delay and start life-saving accurate immunosuppressive treatment.