From this case in which antemortem diagnosis of POEMS syndrome could not be made, we can learn a lesson; namely, “before establishing a clinical diagnosis of idiopathic PH, it is essential for clinicians to rule out the possibility of the PCD-related PH.” For this purpose, it is indispensable to perform serum protein electrophoresis for detection of M protein, to estimate cytokine markers such as VEGF, and to radiologically evaluate spinal bones. Here, VEGFA is linked to POEMS syndrome.