Secondary thrombotic microangiopathy (TMA) can be induced by several underlying conditions and drugs, yet coexistence of TMA and inflammatory bowel disease (IBD) has only infrequently been documented. A successful management beyond supportive care in cases with secondary TMA represents a challenge, as some underlying conditions might amplify complement dysregulation or even unmask a genetic predisposition to atypical hemolytic uremic syndrome (HUS)-both of which could require treatment with a complement blocking agent. We observed a case in which TMA developed in a patient with ulcerative colitis (UC). Genetic screening showed a heterozygous mutation in diacylglycerol kinase ε (DGKE). Eculizumab resulted in complete resolution of TMA, however UC relapsed after cessation of eculizumab.