Vasoactive intestinal peptide (VIP) secreting tumors (VIPomas) are insidious functional neuroendocrine tumors originating mainly from pancreatic islet cells. Hepatic localization is considered exceedingly rare as only few cases have been reported in the literature. Diagnostic and therapeutic management of this tumor is still not clearly codified and therefore represents a real challenge for clinicians. Herein we report a unique case of a primary hepatic VIPoma recurrence in a female patient 22 years after curative resection. The patient had two sessions of transarterial chemoembolization. Complete symptomatic improvement was achieved since the first day after the first session. This case highlights that long-term follow-up for patients with hepatic VIPoma is mandatory as recurrence could occur several years after curative surgical treatment.