Hemimegalencephaly is a rare brain malformation consisting of the enlargement of 1 hemisphere, often associated with abnormal cortical gyration, thick cortex, large neurons, and increased astrocytes. Cranial asymmetry is the first clinical sign usually present at birth; in the most severe cases, hemimegalencephaly may be evident during pregnancy. Hemiparesis, intractable epilepsy, and developmental delay are the typical clinical manifestations. Tuberous Sclerosis Complex is an autosomal dominant disorder affecting about 1 in 6000 live births; the number of spontaneous mutations is remarkable. It is characterized by the development of hamartias, or nongrowing lesions, and hamartomas, which grow as benign tumors and rarely progress to malignancy. These lesions most frequently involve the brain, skin, kidneys, eyes, and heart. The rare association of hemimegalencephaly and tuberous sclerosis complex has been reported in a few cases. The authors report the case of a 4-year-old boy with left hemimegalencephaly, tuberous sclerosis complex genetically confirmed, and intractable epilepsy originating from the nonhemimegalencephalic hemisphere.