Muscular dystrophies and myopathies are inherited disorders that affect the neuromuscular junction.Limb-girdle muscular dystrophy (LGMD) is a rare neuromuscular condition that includes a variety of disorders with heterogeneous causes, as well as mutations within the same gene that can result in different phenotypic expressions. As the natural course of the disease progresses, the risk of surgery increases concomitant with the increased comorbid conditions associated with the later phase of the disease. Notably, perioperative complications do not correlate directly with the severity of the disease, as they can manifest even in patients with mild symptoms. Therefore, it is essential for patients to receive thorough preoperative consultations and evaluations. Factors such as the severity of the condition, age at onset, and specific characteristics of LGMD can differ significantly among individuals.Pregnant women with LGMD are particularly susceptible to obstetric complications, especially if they experience significant weakness in the pelvic girdle muscles and respiratory insufficiency. Symptoms may worsen during pregnancy, leading to increased respiratory compromise. Additionally, the progression of the disease is observed during this period. The various anaesthetic challenges presented by a patient with LGMD complicate the anaesthetic management. However, with thorough pre-operative evaluation and a judicious selection of anesthesia and anaesthetic agents, it can be managed safely.Here we discuss a case of pregnant patient diagnosed with LGMD scheduled for elective caesarean section under spinal anesthesia.