Abstract Follicular lymphoma is an indolent B cells proliferative disorder that represents approximately 35% of all non‐Hodgkin lymphomas. Although spontaneous remission is uncommon in patients with low grade non‐Hodgkin lymphomas, some cases have been reported. We present a case of follicular lymphoma for which we have documented a spontaneous remission both with serial instrumental investigations and through histological biopsy of the bone marrow. The patient is still in remission after 2 years of follow‐up. The causes for a spontaneous remission are not known, and we can only hypothesize a possible reawakening of the host's immune response against the tumour.