We present a case of a pediatric patient presenting with gastrointestinal symptoms found to have acute appendicitis and ultimately diagnosed with acute promyelocytic leukemia (APL). He had severe hemorrhage at presentation, with laboratory abnormalities concerning for disseminated intravascular coagulation, initially attributed to infection. Prior to initiating standard therapy used to treat APL, he required stabilization and numerous blood product transfusions. He was treated with all-trans retinoic acid (ATRA) and arsenic trioxide and ultimately went into remission. We present this case as there is limited literature regarding APL manifesting with gastrointestinal symptoms in pediatric patients.