Primary myeloid sarcoma is a rare extramedullary manifestation of acute myeloid leukemia (AML). Typically, myeloid sarcoma presents after a diagnosis of AML or other myeloproliferative disorder. However, primary myeloid sarcoma may present without any preexisting condition, thereby making it extremely difficult to diagnose. We discuss a case of a 22-year-old female who was misdiagnosed initially with acute appendicitis and underwent an appendectomy. Postoperatively, she continued to be symptomatic and eventually developed small bowel obstruction. Diagnostic laparoscopy revealed multiple small bowel masses as well as diffuse abdominal and pelvic lymphadenopathy. After extensive pathological review and additional workup consisting of immunohistochemistry and molecular studies, the correct diagnosis of myeloid sarcoma was made. This review will discuss the presentation, diagnosis, management, and prognosis of primary myeloid sarcoma.