Achalasia is a rare motility disorder of the esophagus. Of more rarity is its occurrence in pregnancy. The disease is associated with varying degrees of malnutrition, with intrauterine growth restriction and even fetomaternal death reported in worse cases. Its diagnosis is usually confused for hyperemesis gravidarum in early pregnancy, and diagnosis is thus often delayed until the disease is advanced. Its evaluation and treatment pose a challenge due to associated fetomaternal risks of management. A case of achalasia diagnosed in the second trimester of pregnancy was presented. The patient was evaluated and subsequently had laparotomy and modified Heller's esophagocardiomyotomy with a good outcome. Based on a review of the literature and own experience, an algorithm is proposed for its evaluation and treatment in a resource-poor setting.