Congenital methemoglobinemia is a rare disease characterized by cyanosis and a left shifting of the oxyhemoglobin dissociation curve. The disease necessitates avoidance of certain medications commonly used in obstetrics, making labor analgesia and anesthesia challenging. We present a case report of peripartum anesthetic management of a pregnant patient with congenital methemoglobinemia type 1 who received remifentanil labor analgesia and continuous methemoglobin monitoring. Continuous real-time monitoring of methemoglobin concentrations may prove to be a useful monitor in future care settings. A review of literature encompassing various perioperative and obstetric anesthesia and analgesia management considerations is presented.