We report an 11-year follow-up of a case of granulomatous slack skin. The patient was first treated surgically followed by a rapid relapse. Then he was treated by α-interferon during 15 months. When this treatment was stopped, the disease relapsed again. Extensive surgery was undertaken. Recently, a new relapse has occurred which was again treated by extensive surgery. No other manifestation of a lymphoproliferative disorder appeared. The clinical, histological, immunohistochemical, ultrastructural, molecular biological and genetic studies with a review of 39 other cases described in the literature are presented.