Mahvash disease is an autosomal recessive condition that has been described fewer than a dozen times in the literature. Although reported cases are scarce, the prevalence of Mahvash disease, as estimated on the basis of genomic studies, is as high as four cases per million persons.7 Among the known cases, a number of overlapping features have been reported, including abdominal pain, hyperglucagonemia without glucagonoma syndrome, pancreaticomegaly due to α-cell hyperplasia, hyperaminoacidemia, and the development of pancreatic neuroendocrine tumors