Arachnoid cysts are a congenital disorder, and most cases begin during infancy; however, onset may be delayed until adolescence. Here we present an unusual case of atypical Paraplegia with Lymphoedema, with onset during adolescence and rapid progression in a young female patient who showed the characteristic appearance of cyst on magnetic resonance imaging (MRI) of spinal cord. This report intends to highlight paraplegia with lymphoedema, as a rare cause of spinal cord compression in pediatric population along with congenital defect manifestations and an interesting radiology finding of the disease.