The association of colorectal cancer with ulcerative colitis (UC) is well-recognized. However, not much is known regarding the increased risk of extra-intestinal malignancies with UC. Several studies have revealed that patients with UC are at in-creased risk of hepatobiliary cancer, nonmelanoma skin cancer, and myeloid leukemia. A 41-year-old man was diagnosed with UC in 1993 and had three relapses over the next three years. Six months after the fourth remission, he developed pan-cytopenia and was diagnosed with acute promyelocytic leukemia (APL). He achieved complete remissions of APL through chemotherapies, and, since then, no recurrences of APL and UC were observed.