Inflammatory myofibroblastic tumors (IMTs) are a type of visceral non-malignant but invasive mass diagnosed in adolescents. Preferred management based on outcomes is surgical resection. We present a patient with successful non-surgical management due to multifocal IMTs of the lungs. After failing to respond to five combinations of previously first line therapies, rituximab was initiated with complete resolution and durable response. Follow up imaging at 19 months off therapy continue to show complete remission. We successfully treated a patient with multifocal and multiply refractory IMT using rituximab therapy which should be considered as a non-surgical option in the future.