Acquired pure megakaryocytic aplasia (APMA) is a rare disorder in the field of hematological diseases. We report the case of a patient with APMA treated with mycophenolate mofetil and review the literature associated with this. Until today, very few cases of APMA have been reported and the disease etiology still seems to be unclear. There have been no reported cases of treatment of APMA treated with mycophenolate mofetil.