Objective: We report an extremely rare case of family clustering of cholesteatoma.

Method: Case reports and a review of the world literature concerning cholesteatoma and heredity are presented.

Results: The family consists of parents and seven siblings of whom the mother and three sons have been surgically treated for cholesteatoma. All cholesteatomas in the family are acquired and all have a history of otitis media. Cholesteatomas occur with an incidence of 5/100 000 in Greenland, corresponding to two to three new cholesteatoma patients per year among the 57 000 inhabitants of Greenland. The family is very exceptional and interesting for further research concerning heredity in the pathogenesis of acquired cholesteatoma.

Conclusion: To our knowledge this is the first report in the world literature of family clustering of acquired cholesteatoma. This case indicates that hereditary factors interplay with other factors in the pathogenesis of cholesteatoma.