Log-term prognosis of children suffering from high-risk neuroblastomas is characterized by a shortened event-free survival, especially if metastases remain after chemotherapy. We report the case of a 3-year-old boy afflicted with a stage 4 neuroblastoma and persistent residual lymph node metastases despite the administration of a various number of treatment modalities. The insertion of a MIBG (metaiodobenzylguanidine) single-photon emission computed tomography (SPECT)-CT and radio-guided surgery implementing a hand held gamma probe finally allowed the exact localization and resection of the suspected lymphatic tissue. As a consequence, the child has been under event-free remission for 20 months. Because study-based knowledge is missing due to the small number of affected patients, individual case reports are helpful to improve future treatment strategies.