A clinical and histologic study was conducted on 27 Portuguese water dogs with non-inflammatory, regionalized, cyclic hair loss. Histologic findings were compared with biopsies from six normal adult and 25 normal puppy Portuguese water dogs with no known relatives with hair loss. The hair loss in the alopecic dogs was due to a follicular dysplasia with abnormal melanization of the pilosebaceous units. Unique histopathologic findings included prominent apoptosis of keratinocytes in the inner and outer root sheath, and dissolution of the hair matrix of anagen hair follicles. A familial tendency with an early age at onset was noted, suggesting an inherited basis for the dysplasia.