In the PDH group, 36/47 dogs (76.6%) were diagnosed based on an adrenocorticotropic hormone (ACTH) stimulation test (ACTHst) only, 7/47 (14.9%) had a combination of ACTHst and low‐dose dexamethasone suppression test (LDDST), 3/47 (6.4%) had ACTHst and endogenous ACTH (eACTH) assay and 1/47 (2.1%) had ACTHst, LDDST, and eACTH. Here, POMC is linked to pyruvate dehydrogenase deficiency.