Context Cushing syndrome is an uncommon manifestation of multiple endocrine neoplasia type 1 with a possible multifactorial origin pituitary or ectopic adrenocorticothropic hormone producing tumors and adrenal tumors. Adrenocorticothropic hormone producing pancreatic neuroendocrine tumors are also very rare tumors, they represent the less frequent pancreatic neuroendocrine tumor in multiple endocrine neoplasia type 1. Case report We present a forty-four-year-old man with primary hyperparathyroidism, adrenal adenoma, pancreatic cyst and an adrenocorticothropic hormone producing pancreatic neuroendocrine tumor without clinical manifestations. Instead the patient had a stable pancreatic cyst since 6 years, only the laboratory analysis revealed an adrenocorticothropic hormone producing pancreatic neuroendocrine tumors hypercortisolism; (111) In-Octreoscan was able to localize the lesion while a magnetic resonance imagingscan performed the characterization. A germ line mutation that affects the splicing of the Menin gene was detected. After a-cephalic duodenopancreatectomy, the biochemical analysis for hypercortisolism is normal. Conclusion The patient continues asymptomatic and currently he is included in a regular follow up for multiple endocrine neoplasia type 1 syndrome associated manifestations. An appropriate study and follow up of these patients is necessary to warrantee an early diagnosis of neoplastic lesions and improve their quality of life.