Here, we report on a 4-year-old child who presented with an aggressive somatotropinoma harboring a heterozygous aryl hydrocarbon receptor interacting protein (AIP) mutation and who, in addition to surgery and radiotherapy, required a somatostatin analogue, pegvisomant, temozolomide, and bevacizumab therapy to control tumor growth and hormone excess. This evidence concerns the gene AIP and neoplasm.