Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent attacks of fever, peritonitis, pleuritis, arthritis, or erysipelas-like skin lesion. FMF is the most common periodic febrile syndrome affecting more than 150,000 people worldwide. The majority of patients develop FMF before the age of 20. FMF may cause amyloidosis, which mainly affects the kidneys but may also be accumulated in other organs such as the heart, gastrointestinal tract, and reproductive organs. FMF being a systemic disorder with a risk for amyloidosis, affecting patients in their childbearing years, and with its lifelong colchicine therapy raises concern about its effect on the reproductive system. In this article, we review the impact of FMF and its treatment to the reproductive system of male and female patients, pregnancy, and lactation.