BACKGROUND: Comprising less than 1% of all brain tumors in both children and adults, choroid plexus epithelium tumors (CPTs) are uncommon intracranial neoplasms. These tumors demonstrate age-specific characteristics. While typically found in the supratentorial compartment and diagnosed in children\'s first year of life, adult-onset CPTs are exclusively located in the infratentorial region. AIM: This manuscript advances our comprehension of CPT by highlighting the critical role of molecular profiling in guiding individualized treatment strategies. Furthermore, it underscores the limitations of radiotherapy. Recognizing the complexity of these rare tumors, we emphasize the necessity for collaborative research efforts and exploration of new therapeutic approaches to improve patient outcomes for this challenging neurological condition. MATERIAL AND METHODS: Histopathological diagnoses were obtained from surgically resected tumors at Centro Medico Nacional 20 de Noviembre, Mexico City (Department of Neurosurgery). The cohort comprised four children (two females and two males) and three adults (one male and two females). RESULTS: This study retrospectively analyzed data from seven patients diagnosed with CPT over a 5-year period. The pathological distribution consisted of three carcinomas, three papillomas, and one disseminated choroid plexus papilloma. Patient ages ranged from 1 to 62 years. All patients received chemotherapy, with four patients additionally undergoing radiotherapy. The median survival rate was six months, with one patient (carcinoma diagnosis) succumbing to the disease. CONCLUSION: CPT, characterized by low incidence, present a significant clinical challenge. Histological grade remains the primary prognostic factor. Disseminated choroid plexus papilloma, an infrequent entity with limited reported cases, exhibits no response to radiotherapy. Moving forward, this field urgently requires the exploration of targeted molecular therapies and minimally invasive surgical approaches to address these rare and intricate tumors.