Neuroblastoma is the most common extracranial solid tumour in preschool-aged children, with a median age of onset of 2 years. The most frequent site of occurrence is retroperitoneum. An extensive surgical resection is often needed and is an important tool in the management of this disease even in metastatic patients. We describe the nutritional management of a chylous leakage occurred in a toddler with metastatic neuroblastoma after its surgical resection. A multimodal approach including a-lipidic diet and parenteral nutrition allowed both chylous fistula closure and specific nutritional requirements coverage without impairment of neither oncological treatment following surgery nor child's growth.