Guillain-Barre syndrome (GBS) is rarely reported in children with acute lymphoblastic leukemia (ALL) and may be difficult to differentiate from vincristine induced neuropathy. Only few case reports highlighted GBS with ALL. We report a 10-year-old male child who was a diagnosed case of ALL since 3 month on chemotherapy. At 3(rd) week of chemotherapy, he developed rapidly progressive ascending motor quadriparesis over 2 days. Clinical and electrophysiology revealed acute motor axonal neuropathy (AMAN) variant of GBS. He was treated with intravenous immunoglobulin (2 g/kg) without discontinuing chemotherapy. Complete recovery took 12 weeks despite immunotherapy, and it was corroborating to slow remission. We concluded that AMAN variant is usually present in B-cell type ALL, may be causal for GBS and it takes 6-16 weeks to complete recovery which may correspond to remission of ALL. However, it needs to be studied. We also present a meta-analysis of previously reported cases of GBS in ALL.