Background: Merosin positive congenital muscular dystrophy poses significant challenges for anesthesiologists due to its low prevalence and limited research available. Additionally, there is a risk of complications arising during surgical procedures. Given the scarcity of literature on anesthetic management in patients with this condition, it becomes crucial to share previous successful experiences in handling such cases. Case Report: We present the case of a 6-year-old child diagnosed with merosin-positive congenital muscular dystrophy who underwent bilateral congenital cataract correction under general anesthesia. In this particular case, neuromuscular blockade was not employed, and instead, bilateral ocular peribulbar block was administered. Conclusion: This article aims to provide an overview of the anesthetic management of a child with a rare congenital muscular disease, highlighting the potential risks of peri-operative complications such as cardiorespiratory failure, rhabdomyolysis, and hyperkalemia.