Background <p>Neurofibromatosis type 1 is an autosomal dominantly inherited disorder caused by pathogenic variants in the <i>neurofibromatosis type 1</i> gene, resulting in a predisposition to multiple tumors. Typical neurofibromatosis type 1-associated tumors include cutaneous and plexiform neurofibromas, optic pathway gliomas, breast cancer, and malignant peripheral nerve sheath tumors. The aim of the study was to report a rare case with multiple neurofibromatosis type 1-unrelated tumors in addition to neurofibromatosis type 1-related ones.</p> Case presentation <p>A 36-year-old Caucasian female patient with neurofibromatosis type 1 had a rare tumor spectrum including ganglioglioma, malignant teratoma, pheochromocytoma, vestibular schwannoma, and meningioma in addition to typical neurofibromatosis type 1 tumors such as cutaneous and plexiform neurofibromas. A pathogenic variant in the <i>neurofibromatosis type 1</i> gene (c.2446C &gt; T, p.Arg816*) was identified in blood-derived DNA while no pathogenic variant was found in the <i>neurofibromatosis type 2</i> gene.</p> Conclusion <p>Though rare, multiple neurofibromatosis type 1-unrelated tumors can develop in neurofibromatosis type 1 patients, which demands attention and interdisciplinary management. All related genes should be screened for potential pathogenic variants.</p>