BACKGROUND Here, we report an atypical HIV-vacuolar myelopathy and search the available medical literature about atypical presentations of human immunodeficiency virus associate vacuolar myelopathy (HIV-VM) and immunoglobulin therapy response. METHODS A 26-year-old lady who was 4 weeks postpartum presented to us with acute flaccid quadriparesis, with no sensory level. Extensive workup ruled out other causes of myelopathy. She developed a stage 3 acute kidney injury, and MRI showed diffuse cord atrophy involving the lower cervical and thoracic cord. The patient received IV-immunoglobulin, ARVs, and supportive therapy with inadequate response. Unfortunately, she developed nosocomial pneumonia and died. CONCLUSIONS In HIV-VM, there is spinal cord atrophy, which mainly involves the thoracic cord. In our case, this pathological process also affected the spinal cord's cervical region, leading to flaccid tetraplegia, with high CD4 level, without response to the treatment, including intravenous immunoglobulin. Vacuolar myelopathy, HIV, Immunoglobulin therapy, flaccid tetraplegia, hypokalaemia. Renal failure.