Subependymal giant cell astrocytoma (SEGA) is the most common central nervous system tumor in patients with tuberous sclerosis complex (TSC). SEGAs are generally benign, non-infiltrative lesions, but they can lead to intracranial hypertension, obstructive hydrocephalus, focal neurologic deficits, and even sudden death. Surgical resection has been the standard treatment for SEGAs, and it is generally curative with complete resection. However, not all SEGAs are amenable to safe and complete resection. Gamma Knife stereotactic radiosurgery provides another treatment option as a primary or adjuvant treatment for SEGAs, but it has highly variable response effects with sporadic cases demonstrating its efficacy. Recently, biologically targeted pharmacotherapy with mammalian target of rapamycin (mTOR) inhibitors such as sirolimus and everolimus has provided a safe and efficacious treatment option for patients with SEGAs. However, SEGAs can recur few months after drug discontinuation, indicating that mTOR inhibitors may need to be continued to avoid recurrence. Further studies are needed to evaluate the advantages and adverse effects of long-term treatment with mTOR inhibitors. This review presents an overview of the current knowledge and particularly highlights the surgical and medical options of SEGAs in patients with TSC.