A 39-year-old woman was admitted to hospital due to perforated relapsing duodenal ulcer. Clinical, laboratory, and surgical examinations revealed a peripancreatic lymph node gastrinoma as the cause of Zollinger-Ellison syndrome. Further examinations established multiple endocrine neoplasia type 1 (MEN1) with a germline mutation at codon 1153 (T->A) in exon 7, causing an amino-acid change, from isoleucine to asparagine (Ile348Asn), in the MEN1 gene. The following findings strongly supported a diagnosis of primary lymph node gastrinoma: a rapid fall of the serum gastrin level after operation, the continuous normalization of the serum gastrin level before and after secretin stimulation, the lack of any symptoms, and the absence of another tumor for 13 years after surgical resection of the tumor-bearing lymph node. A review of similar cases in the world literature reveals that not all gastrinomas in lymph nodes are the result of metastastic spread. A long-term symptom-free follow-up after the excision of a lymphnode gastrinoma is the only reliable criterion for the diagnosis of a primary lymph node tumor. To our knowledge, this is the only well-documented case of a primary lymph node gastrinoma in a patient with MEN1. Our case supports the idea that any gastrinoma in patients with MEN1 should be surgically resected for cure if possible.