Stickler syndrome, a genetic disorder characterized by collagenopathy involving the craniofacial, skeletal, ocular, and auditory organs, is inherited in an autosomal dominant manner. We reported a 7-year-old boy diagnosed with Stickler syndrome type I (STL1) presenting with profound bilateral sensorineural hearing loss along with craniofacial, skeletal, and ocular abnormalities. © The Author(s).