Objective: To evaluate the location of rhabdomyomas in the heart, and the spontaneous regression, clinical and echocardio- graphic findings and association of rhabdomyomas with tuberous sclerosis. Materials and Methods: The medical files of 12 rhabdomyoma cases diagnosed between 2005 and 2011 in the outpatient clinic of Paediatric Cardiology Department were retrospectively evaluated. Rhabdomyoma diagnosis was based on transthoracic echocardiography (TTE) and tuberous sclerosis was diagnosed according to clinical characteristics and imaging methods. Results: The mean age at diagnosis of 12 cases, eight (66.6%) male, four (33.3%) female, male/female ratio 2, was 3.3+4.3 years (3 months-13 years). Seven cases (58.3%) were diagnosed to have definite tuberous sclerosis. Location of rhabdomyomas was as follows, seven cases (58.3%) in the left ventricle, two cases (16.6%) in the right ventricle, two cases (16.6%) in both ventricles and one case (8.3%) in the right atrium. The mass showed spontaneous regression in four of our cases (33.3%). Left ventricular size and systolic functions were normal in all cases. While the majority of the cases were asymptomatic, three cases had signs of congestive heart failure and one case had arrhythmia. The tumours of the cases with congestive heart failure were surgically excised. Conclusion: Consistent with the literature, the frequency of definite tuberous sclerosis was 58.3%. While most of the rhabdo- myomas were located in the left ventricle, 4 (33.3%) cases had spontaneous regression.