OBJECTIVE: To determine the incidence, etiology and types of orofacial clefts (FOFs) in a sample of patients with congenital heart disease (CC). METHODS: The sample consisted of patients hospitalized for the first time in the intensive care unit of a pediatric hospital. Clinical data were obtained by completing a standard protocol. We carried out the high-resolution karyotype and made to search for 22q11.2 micro deletion by the technique of fluorescent in situ hybridization (FISH). RESULTS: The sample consisted of 297 patients, 151 males, aged 1 day to 13 years (59% <1 year). FOFs were found in 5 patients (1.7%) (Ranged from unilateral cleft lip by unilateral cleft lip associated with cleft palate). The CC further observed among patients with FOFs was septal defect (n = 2). Three patients were syndromic and chromosomal abnormalities were observed in 1 (deletion of 6p). Had a phenotype of oculo-auriculo-vertebral, with gestational history of epilepsy and the use of Phenobarbital. There were no patients with 22q11 deletion in the study. CONCLUSIONS: In the literature, we found only a few studies linking the frequency of FOFs in patients with CCs. However, these are records (based on the database), non-specific, since the frequency change extra cardiac rate generally between individuals with CC. The 22q11 deletion syndrome is also a common abnormality in patients with these findings. However, in our study none of them was identified with the amendment. The finding of FOFs can interfere with the management of patients with CC.