Tuberous sclerosis complex (TSC) is associated with significant psychiatric comorbidity mainly in the form of autistic disorders, hyperkinetic disorders, depression, and anxiety disorders. There are very few reports of bipolar disorder in TSC. The authors present the case of a patient with TSC having bipolar disorder manifesting as manic as well as depressive episodes. The diagnosis of TSC was based on the presence of facial angiofibromas, enamel pits, and shagreen patches on clinical examination, and the presence of cortical tubers and calcified subependymal nodules on neuroimaging. The patient presented during the manic phase of the illness and responded well to treatment with carbamazepine and haloperidol and became symptom free within 1 month. The patient's neuropsychological assessment revealed a borderline level of intellectual functioning and deficits in attention and concentration, recent memory, delayed and immediate recall, and visual retention. To our knowledge, this is the first report of TSC with bipolar disorder having both manic as well as depressive episodes. The nature of association between TSC and bipolar disorder, and its clinical implications are discussed.