OBJECTIVE: To present the case of congenital a carrying child of cholesteatomatous whose father and paternal uncle had been submitted the surgery for the treatment of this disease. CASE REPORT: JFSS, ten years of age, masculine sex, white. The pediatrician observed to the otoscopy mass off-white behind the left eardrum membrane. Directed for evaluation with the otolaryngologist. Familiar antecedents: submitted father and paternal uncle to the surgery for the congenital treatment of cholesteatomatous to the 9 and 6 years of age respectively. Computerized Tomography (TC) disclosed to the presence of mass in meson-eardrum and left hypo-eardrum, around of the chain to ossicular. The patient was submitted excision of cholesteatomatous for access to retroauricular trans-channel. Fistula in the promontory was observed. The patient find itself in 2th year of postoperative without topographic evidences of recurrence of the disease and with preservation of the hearing in the operated ear. CONCLUSION: We consider with this case report to present plus a possibility for the controversial etiological diagnosis of congenital cholesteatomatous.