Purpose: The purpose of this descriptive study was to describe oral manifestations and oral health quality of life (QOL) in children aged six months to six years, 11 months with tuberous sclerosis complex (TSC). Methods: Parents of participants completed an oral health QOL survey. The presence of oral lesions associated with TSC was assessed. Results: Sixty-two participants enrolled between February 2015 and March 2017. Among the significant oral health findings, 11 (17.7 percent) had enamel pitting, 33 (53 percent) had no previous dental examination, eight (12.9 percent) did not brush their teeth, and eight (12.9 percent) had caries present. Per the QOL survey results, 18 (31 percent) had difficulty sleeping, 22 (37.3 percent) experienced irritability/frustration, and 15 (29.4 percent) had difficulty pronouncing words. Developmental delays were present in 40 (70.2 percent), seizures occurred within the last three months in 37 (59.7 percent), and a mammalian target of rapamycin inhibitor medication was used for 22 (35.4 percent). Conclusions: Enamel pitting was observed in 17.7 percent of children in the primary dentition. Oral health quality of life in this tuberous sclerosis complex cohort was significantly reduced.