Inner ear malformation accounts for approximately 20% of reported cases of congenital sensorineural deafness. It results from developmental arrest in different stages of embryogenesis. Aplasia of one of the semicircular canals with or without cochlear malformation is well known and has been reported in journals. However; to our knowledge, aplasia of two semicircular canals have been rarely reported, and for this reason the clinical reflection of this anomaly is poorly understood. This case will be hopefully used to gain an in-depth understanding of illness and help in the diagnosis and proper management of future cases. In this paper we present a report concerns a 2-year-old boy with no family history of hearing loss, presenting with bilateral profound sensorineural hearing loss with no other congenital anomalies. CT scan and MRI shows dysplastic bilateral vestibules, bilateral hypoplastic cochlea and bilateral lateral and posterior semicircular canal (SCC) aplasia. Keywords: Semicircular canal aplasia, sensorineural hearing loss, CT scan, cochlear hypoplasia