We retrospectively analysed the clinical and CT findings of 24 cases with tuberous sclerosis with specialemphasis upon the atypical presentation. The cases with classic clinical triad were accounted for in 21%, while 33% presented with atypical clinical features. The most common and characteristic CT finding is that of thesubependymal calcified nodules of the lateral ventricle, which was seen in 85% of our cases. Cortical tuber andwhite matter lesions were demonstrated in 65% and 55%, respectively. Three cases showed cortical tuber usuallyexhibited low density or calcified lesion, but showed high density in 2 cases, Other findings of tuberoussclerosis included intraventricular tumor, ventriculomegaly, and cortical atrophy. One case clearly demostratedparenchymal tuber on MR imaging. In conclusion, diagnosis of tuberous sclerosis is usually made on the clinicalbases initially. However, CT and MR are also diagnostic even in unusual clinical presentation such as unexplainedretardation or epilepsy or when the classic clinical triad is incomplete. If only cortical tuber or white matterlesion is present, it is difficult to diagnose tuberous sclerosis and to differentiate from other cortical masslesion. Therefore, careful evaluation is required with familarity with the atypical appearance of the disease.