Spontaneous intracranial hypotension, typically resulting from spontaneous leakage of spinal cerebrospinal fluid (CSF), is an uncommon and underrecognized condition, particularly during pregnancy. Diagnosis can be challenging when cranial imaging is normal, and presentations in very early pregnancy are sparsely reported. This report describes the case of a 33-year-old woman (gravida 2, para 1) who presented at 4 weeks and 4 days of gestation following frozen embryo transfer with a severe orthostatic bi-occipital headache associated with nausea, vomiting and neck pain. There was no preceding trauma or recent spinal instrumentation. Magnetic resonance imaging (MRI) of the brain was unremarkable; however, whole-spine MRI revealed an extensive longitudinal CSF-signal collection extending from the cervical to the lumbar spine, compatible with spinal CSF leakage. Three epidural blood patches produced partial but progressive symptomatic improvement. Residual symptoms were managed conservatively with hydration, caffeine, analgesia, and relative activity restriction, with eventual complete clinical resolution. The remainder of the pregnancy progressed to term without recurrence, and the patient underwent a repeat cesarean delivery under general anesthesia in accordance with maternal preference. Spontaneous intracranial hypotension should be considered in pregnant patients presenting with new-onset orthostatic headache. Normal brain MRI does not exclude the diagnosis, and spinal MRI may demonstrate evidence of CSF leakage when clinical suspicion remains high. This case highlights that spontaneous spinal CSF leakage may present in very early pregnancy, following a medicated frozen embryo transfer, an infrequently reported clinical scenario. Management should be individualized and multidisciplinary, given the limited pregnancy-specific evidence and variable response to epidural blood patching.