Darier-White disease (keratosis follicularis) is a rare autosomal dominant genodermatosis characterized by hyperkeratotic papules and plaques in seborrheic areas, often presenting with nail abnormalities and occasionally mucous membrane changes.It has been associated with neurocognitive/psychiatric disorders, ankylosing spondylitis, hidradenitis suppurativa and nephritis. Affected individuals also have an increased tendency to cutaneous infections. However, a thorough literature search showed no association with sensorineural hearing loss. We herein report the case of a 34-year-old Nigerian man with disfiguring Darier-White disease (DWD) associated with spondyloarthropathy and sensorineural hearing loss. We affirm that early diagnosis, prompt and appropriate therapy and adequate patient education can avert undesirable outcomes.