Multiple endocrine neoplasia (MEN) is a group of rare hereditary syndromes characterized by increased risk of developing multiple endocrine and nonendocrine tumors starting from a young age. Two main types of MEN syndrome include MEN1 and MEN2, which differ in etiology and clinical presentation. MEN is caused by gene mutations that lead to tumorigenesis. MEN1 is caused by MEN1 gene mutation and MEN2 by RET gene mutation. The mutations are inherited in an autosomal dominant manner, although some patients present with de novo mutations. Tumors that typically arise in the setting of MEN1 include parathyroid tumors, duodenopancreatic neuroendocrine tumors and pituitary adenomas. The most common tumor in MEN2 patients is medullary thyroid carcinoma, but pheochromocytoma, parathyroid tumors and other nonendocrine manifestations can also occur. The clinical presentation of MEN syndromes is heterogeneous, thus making it challenging to diagnose. Making diagnosis requires clinicians’ knowledge and experience as well as a wide range of diagnostic methods that first include genetic testing accompanied by measuring various endocrine and biochemical markers and using imaging methods. Genetic testing has a significant role in confirming the diagnosis. It should be offered to individuals presenting with typical clinical manifestations of each syndrome as well as to family members that are at risk of inheriting the mutation. Prospective genetic testing in asymptomatic individuals with a positive family history is of a great importance and enables an early recognition of mutation carriers together with starting an adequate follow-up and a prompt treatment when needed. The treatment of MEN is often challenging, and it includes surgical resection of the tumors, symptomatic treatment of hormone hypersecretion in addition to other therapeutic modalities such as chemotherapy, radiotherapy, and targeted therapy. Due to the course of MEN syndromes, the patients require multidisciplinary approach and a life-long surveillance. We also presented case reports of two patients with different types of MEN syndrome. We pointed out the specifics of each syndrome type that occurred in these patients along with the results of genetic testing, remaining diagnostic work-up, treatment and further surveillance plan.