Myelodysplastic syndrome (MDS) with basophilia is a rare condition and has yet to be classified under the 2016 revision to the World Health Organization classification of myeloid neoplasms. However, few reports have described the prognostic significance of basophilia in MDS. Here, we report a case of a 60 year old male who was admitted to the hospital with complains of generalised body weakness, easy fatiguability and breathlessness on mild exertion for one month. Initial investigations showed features of pancytopenia with basophil 12% on complete blood count (CBC). Reticulocyte count was less than 0.5%.Bone marrow aspiration (BMA) showed myeloid and erythroid series cells with features of dysmyelopoiesis and severe degree of dyserythropoiesis. Quadrinucleate, pentanucleate and gigantoblasts forms of erythroblasts were also noted. These findings led to the diagnosis of MDS with basophilia, but patient died within two months of diagnosis showing its poor prognosis. To increase awareness of the prognostic significance of MDS with basophilia, we report a case of MDS with basophilia which is a rare entity and showed poor prognosis.