Background <p>Subependymal giant cell astrocytoma (SEGA) is a rare pediatric tumor that most commonly occurs in the setting of tuberous sclerosis complex (TSC). The deep anatomical location of these lesions makes the selection of an appropriate neurosurgical approach critical. However, minimally invasive techniques have emerged as a safe and effective alternative for the surgical resection of tumors located in deep regions.</p> Observations <p>A 7-year-old male with a history of TSC presented with a large intraventricular mass located in the frontal horn of the right lateral ventricle, associated with symptoms suggestive of intracranial hypertension. A minimally invasive transcortical approach using a tubular retractor was initially performed, achieving a partial resection of the tumor, then followed by adjuvant targeted therapy and radiotherapy. Due to persistent seizures, the patient underwent a second intervention using a similar transcortical approach and tubular retraction, this time aided by neuronavigation. An estimated extent of resection (EOR) of 90–95% was achieved resulting in improvement of the patient’s neurological involvement.</p> Lessons <p>The integration of minimally invasive tubular retractor systems with neuronavigation represents an effective and safe strategy for the surgical management of SEGAs located in critical intraventricular regions. In this case, neuronavigation was essential in achieving GTR while minimizing intraoperative bleeding and reducing injury to critical cortical and subcortical structures. Minimally invasive approaches proved to be safe and were associated with a favorable postoperative course.</p>