Introduction: Rhabdomyomas are the most prevalent benign heart tumors in the fetal stage and during childhood. Objective: Our objective is to make known our experience over 39 years in pediatric patients with rhabdomyoma in our institution, as well as to emphasize the importance of its detection, study and control due to the association it has with tuberous sclerosis (TS). Materials and methods: We conducted a retrospective, descriptive, and cross-sectional study, from January 1980 to March 2018. Twenty-four met our criteria; we collected information regarding the clinic and cabinet studies, as well as their evolution and the treatment given. They were called by telephone to know their evolution and current status. Results: We found 51 patients with a diagnosis of cardiac tumor, of which 24 were rhabdomyomas. The diagnosis was made prenatal in eight patients, five at birth, and in 11 during the 1 st year of life. The most frequent clinical manifestations were the presence of murmur, arrhythmias, cyanosis, dyspnea, and diaphoresis. In 17 of them a diagnosis of TS was made. Half had follow-up by neurology, ten by dermatology, eight by ophthalmology, and four with genetics. Half were left under surveillance, seven were given medical treatment and five required surgery. Regarding the clinical evolution 17 patients remained stable, five presented spontaneous regression and two died. Conclusions: Rhabdomyoma is a rare benign tumor, its evolution can be malignant and associated with TS, it darkens the prognosis.