A Case of Congenital Methemoglobinemia: Rare but Real

Methemoglobin (MetHb) is a form of hemoglobin in which iron in Hb is in an oxidized form (ferric) instead of ferrous, making it difficult to bind with oxygen. Usually, MetHb is present in small quantities (<1%) in humans, but once MetHb increases beyond 3%, the condition is known as methemoglobinemia. It can be further classified into hereditary and acquired. Hereditary forms are a rare cause of hypoxia and cyanosis. Only a few cases have been reported worldwide. Here, we present a case of a 33-year-old female with congenital methemoglobinemia who remains relatively healthy in spite of her underlying condition. This case report focuses on knowledge sharing and practical aspects of managing patients with congenital methemoglobinemia.