Chest wall tumors in childhood are major challenges with respect to diagnostic workup and treatment. Incidence rate is less than 1 per 1,000,000 and various benign and malignant diseases are noted. From the malignant diseases, Ewing tumors and rhabdomyosarcoma tumors are most often seen. Depending on diagnosis, staging, and age, therapy has to be tailored for each patient, which should be discussed in a multidisciplinary team setting. Radical resection is in most cases the major component of treatment. Use of chemotherapy depends on the diagnosis. In soft-tissue tumors, previously considered to be chemotherapy insensitive, favorable results are currently reported. The role of radiotherapy is debated owing to its pronounced late sequelae in children, but should be added when adequate margins cannot be achieved by surgery.