BACKGROUND: Pancreatic neuroendocrine tumors (PNETs) are rarely found and the majority of PNETs are nonfunctioning. At present, a patient with pancreatic neuroendocrine tumor (PNET), lymphoma, and squamous cell carcinoma (SCC) occurring simultaneously has not been reported. We presented all these three primary cancers in a same patient. CASE PRESENTATION: A 51-year-old male presented with a right testicular mass for four months. He underwent right orchidectomy, and the patient was diagnosed malignant diffuse large B-cell lymphoma. Consequently, CHOP regimen and intrathecal methotrexate were given as treatment. During the metastasis work up, a hypodense lesion at the tail of pancreas was found. Distal pancreatectomy and splenectomy were completed, and the pathological report revealed PNET. Eighteen months later, the patient developed a symptom of odynophagia, direct laryngoscopy was performed and a mass at the left arytenoid was found. The biopsy was done and confirmed the diagnosis of SCC. The patient went through concurrent chemo-radiation with a complete response. Currently the patient remains in a complete remission of all three cancers. DISCUSSION: As the patient had non-functioning PNET and was symptom-free, the diagnosis would have not been discovered without the metastasis workup of lymphoma. Although non-functioning PNET less than 2 cm can be safely observed, the tumor was 4.3 x 1.3 cm and adenocarcinoma was suspected, so the patient underwent the surgery. CONCLUSION: Although many cases with multiple primary cancers have been published, a case of patient with PNET, lymphoma, and SCC occurring as primary cancers has not been yet reported. 2019 The Author(s). Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd.