Subependymal giant cell astrocytoma (SEGA) is the most common tumour seen in tuberous sclerosis complex (TSC). It is a slowly growing grade 1 tumour of glioneuronal origin, usually seen in the first two decades of life. It is seen in lateral ventricles and foramen of Munro and rarely, the third ventricle. There are also tumours seen in multiple organs of the body including kidney, heart, eyes, lung and skin. SEGA is considered as pathognomic of TSC. Adequate surgical resection is the main line of management. Adjuvant radiotherapy (RT) and mTOR inhibitors (Everolimus) have been shown to be of particular value. Herein, we present a case of SEGA of the lateral ventricle in a 17-year old boy with TSC. He had facial nevus, although IQ was normal. After surgery, he was started on conformal RT. RT has increasing role in SEGA and is useful in cases with subtotal resection, residual or recurrent disease. Regular follow-up and imaging after treatment can result in further improved survival.
