Background and aim. Vasoactive intestinal peptide tumor is a rare neuroendocrine neoplasm which causes voluminous watery diarrhea via hypersecretion of electrolytes and water from the intestinal mucosa through a vasoactive intestinal peptide-mediated, cyclic AMP-dependent mechanism. The acid base imbalance generated by the loss of water and electrolytes leads to severe dehydration and potential renal failure, which can ultimately result in death if left untreated. This paper aims to review the clinical, histological, radiological, and diagnostic features of this disease as well as the therapeutic modalities in treating this condition. Methods. A review of literature was performed using MEDLINE, Pubmed, and Cochrane databases in collection of data using MeSH terms including vasoactive intestinal peptide, VIPoma, and WDHA. Results and conclusion. Vasoactive intestinal peptide tumor is a rare neoplasm associated with significant morbidity and mortality through secretion of water and electrolytes in the gastrointestinal tract. The nonspecific clinical presentation of this neoplasm can pose diagnostic challenges, as these tumors can be easily misdiagnosed as other conditions, ranging from laxative overdose to the presence of a carcinoid secreting tumor. Nevertheless, a number of imaging and laboratory studies can facilitate the correct evaluation and diagnosis of VIPoma. Following proper diagnosis, VIPomas are treated by either medical or surgical modalities depending on the existence and extent of metastasis.