Background: Conductive hearing loss secondary to congenital malformation of ossicular chain is rare, diagnosis and surgical treatment are challenging. Aims: To study clinical, audiometric and CT scan characteristics and to evaluate result of ossiculoplasty. Patients: 7 patients suffering from conductive hearing loss associated with congenital malformation of ossicular chain. Methods: Descriptive case study. Results: Pre-operative air-bone gap (ABG) was 46 dB. Abnormalities were'absence of long process of incus in 7/7 cases. Malformation of stapedial supertructure found in 6/7 cases. Modified stapedectomy was employed in 5 cases, another 1 case with malleusfootplate prothese and 1 case with autologous incus interposition. Closure of air-bone gap within 15 dB was achieved in all 7 of cases. Conclusions: Abscence of long process of incus and malformation of stapedial superstructure are most encountered. Diagnosis based on clinical, audiometric presentation, CT scan and intraoperative findings. Significant hearing gain makes ossiculoplasty a valuable treatment.