The development of tumors within the first month of life is extremely rare and this very young age at presentation should raise concern for an underlying cancer predisposition syndrome. This chapter discusses genetic predisposition to neonatal solid tumors, specifically those involving the eyes, kidneys, liver, adrenal glands and lungs. For each tumor type, we discuss the most common associated predisposition syndromes and for eye and lung tumors, we also review the approach to diagnosis and management. The reader is referred to the other chapters of this volume for an overview of management details for the other tumor types. The management of neonates with cancer predisposing syndromes is challenging, with many patients demonstrating multisystem involvement. Accordingly, it is critical to use a team approach, where providers with expertise in neonatology, oncology, genetics, surgery and other pediatric subspecialties can appropriately tailor management of the patient and coordinate genetic evaluation of the family.