Generalized granuloma annulare (GGA) is a rare variant of granuloma annulare, presenting with diffuse papules and annular patches on the trunk and extremities. Though the pathogenesis of GGA has not been totally understood, recent studies have shown the relationship between malignancy and GGA. A 74-year-old man developed a generalized, mildly pruritic erythematous papules, which slowly coalesced into annular patch with elevated rim. Punch biopsy showed palisading and interstitial granuloma with giant cells. A complete blood cell count with differential revealed leukopenia and subsequent findings of the bone marrow biopsy were compatible with myelodysplastic syndrome (MDS). As there was no more progression of MDS, only the skin lesions were treated with hydroxychloroquine, isotretinoin and phototherapy, and improved after 14 months of treatment. We recommend that careful evaluation of the underlying solid tumor, as well as hematologic malignancy, should be done when the dermatologists encounter with the patient presenting generalized granuloma annulare.