Inner ear barotrauma with rupture of the round or oval window secondary to diving and decompression sickness (DCS) of the inner ear can be a difficult diagnosis to differentiate. The dive profile or associated elements of DCS will often confirm the diagnosis. Occasionally, diagnosis is made during recompression or during operation. The differential diagnosis is important, since immediate recompression is indicated for inner ear DCS, while it is contraindicated in cases of inner ear barotrauma. We have found no cases reported in the world literature in which both diseases have been diagnosed and proven simultaneously. We present a case of a diver who developed DCS with inner ear manifestations complicated by a round window fistula. Treatment and clinical outcome are discussed along with a brief review of the suspected cause. © 1990, SAGE Publications. All rights reserved.