The advent of lenalidomide has fundamentally changed the treatment of patients with myelodysplastic syndrome (MDS) and deletion of the long arm of chromosome 5 (del(5q)), inducing high rates of red blood cell transfusion independence and cytogenetic response. We report a case of an 84-year-old patient with International Prognostic Scoring System (IPSS)-defined low-risk MDS with isolated del(5q), who has received continuous lenalidomide treatment for the past 8 years and whose treatment is ongoing. Grade 3 or 4 neutropenia and thrombocytopenia, which occurred in approximately 60 % of the patients during the first weeks of treatment, did not occur in our patient. Also, there were no thromboembolic events or a progression to acute myeloid leukemia. This case highlights the role of lenalidomide in modifying the course of low-risk del(5q) MDS in long-term responders by inducing a durable hematologic response and thereby contributing to control iron overload and improving long-term outcomes in selected patients.