Background: Charcot-Marie-Tooth Disease type 2 (CMT2) is a genetically heterogeneous hereditary motor and sensory neuropathy characterized by axonal degeneration. Diagnosis is established through a combination of clinical history, physical examination, electrophysiological studies, and genetic testing. Case Presentation: We report a case involving a 9-year-old female patient who presented with a two-year history of frequent falls and clumsy gait. Neurological examination revealed a high steppage gait, bilateral foot drop, pes cavus, absent ankle jerks, and impaired dorsiflexion and eversion of both feet. Romberg’s sign was positive. There was no involvement of proximal lower limbs, upper limbs, or sensory modalities. Nerve conduction studies and electromyography demonstrated severe axonal neuropathy confined to the distal lower extremities. Genetic analysis via a neuromuscular gene panel identified a novel de novo heterozygous variant (c.262A>C) in the MFN2 gene, consistent with Charcot-Marie-Tooth Disease type 2A2A. Conclusion: This case highlights a rare de novo MFN2 mutation in a pediatric patient with CMT2A2A, underscoring the importance of early recognition and genetic testing in atypical presentations of hereditary neuropathies.