RATIONALE:: Metastatic neuroendocrine neoplasms (NENs) to the breast are very rare entities comprising only 1% to 2% of all metastatic breast tumors. In this article, we describe a case of a neuroendocrine ileal neoplasm metastatic to breast and liver, with breast metastatic tumor to be the initial manifestation of the disease. PATIENT CONCERNS:: We herein report a rare case of a female patient admitted to our department with a palpable painful mass on her left breast. DIAGNOSIS:: The surgical and histological investigation revealed a metastatic neuroendocrine neoplasm to the breast originated from terminal ileum. INTERVENTIONS:: A left lumpectomy, right hemicolectomy, cholecystectomy, left hepatectomy along with liver metastasectomies (V, VI, VIII) plus radiofrequency ablation of lesions to the right liver lobe plus standard lymphadenectomy was performed. OUTCOMES:: Considering the advanced stage of the disease, the patient received an adjuvant therapy of somatostatin analog plus everolimus. Under the guidance of oncological consultation, patients follow-up with CT and MRI scan and clinical re-evaluations in the first 3 and 6 months, substantiates no evidence of recurrence and she presents herself asymptomatic. LESSONS:: An appropriate level of suspicion and selective immunohistochemistry in these cases, particularly where no prior history of a known primary neuroendocrine neoplasm occurs, may help to diagnose a previously undetected neuroendocrine tumor elsewhere in the body and provide guidance for the appropriate treatment selection.