Ga-DOTATATE PET/CT was performed to determine the cause of endogenous Cushing syndrome in a 10-year-old boy whose MRI studies did not reveal pituitary or adrenal gland abnormality. The PET/CT images demonstrated an intense activity in a small soft tissue nodule in the left upper abdomen where splenules are commonly located. Heat-damaged Tc-labeled red blood cell imaging showed that this soft tissue nodule did not have increased red blood cell activity, which indicated that this lesion was a true somatostatin receptor-rich lesion. The pathology result following surgical resection of the lesion confirmed adrenocorticotropic hormone-producing neuroendocrine tumor.