VIPoma syndrome, a rare neuroendocrine tumor characterized by watery diarrhea, hypokalemia, and achlorhydria, poses a significant clinical challenge due to its life-threatening symptoms and limited treatment options. We present the case of a 44-year-old female with VIPoma, initially unresponsive to standard therapies such as somatostatin analogs and chemotherapy. Despite a challenging treatment course, including loperamide, glucocorticoids, metoclopramide, carboplatin-etoposide chemotherapy the patient’s symptoms persisted. The turning point came with the initiation of sunitinib therapy, leading to a rapid and complete resolution of refractory diarrhea, renal insufficiency, hypotension, and electrolyte imbalances. This case underscores the importance of considering sunitinib as a life-saving treatment in VIPoma patients with resistant symptoms. As VIPoma is a rare entity, collaborative multi-center studies are crucial for enhancing our understanding of its natural course, treatment strategies, and prognosis.