BACKGROUND: Differential diagnosis of acute abdomen in pregnant patients is one of the greatest challenges for the clinician. Occurrence of Familial Mediterranean Fever (FMF) paroxysm of peritonitis and acute cholecystitis during pregnancy is a unique clinical entity that leads to serious diagnostic and therapeutic dilemmas.CASE REPORT: We present the case of a 33-year-old Armenian patient at 16 weeks' gestational age with a history of FMF, who was admitted twice within 1 month with acute abdomen. The first episode was attributed to FMF and successfully treated conservatively with colchicine. The second episode was diagnosed as acute cholecystitis and led to emergent laparoscopic cholecystectomy and lysis of peritoneal adhesions from previous FMF attacks. The patient presented an uneventful postoperative clinical course and had a normal delivery of a healthy infant at the 39th week of gestation.CONCLUSIONS: Pregnant patients with acute abdomen should be evaluated with open mind. To the best of our knowledge, this is the first published report of the coexistence of 2 different causes of acute abdomen during pregnancy. Meticulous history and thorough physical, laboratory, and radiologic examination are the keys to reach a correct diagnosis. Treatment of pregnant patients with acute abdomen should be individualized. Administration of colchicine should be continued during conception, pregnancy, and lactation in patients with FMF history. Laparoscopic intervention in pregnant patients with surgical abdomen such as acute cholecystitis is the optimal method of treatment.