A six-year-old male cocker spaniel was presented to the Veterinary Medical Teaching Hospital, University of Florida, with a three-week history of generalised weakness and myalgia. Electrodiagnostic evaluation, cerebrospinal fluid analysis and thoracolumbar myelography were unremarkable. Biopsies from vastus lateralis and triceps muscles revealed numerous large lipid droplets within type 1 fibres and to a lesser degree within type 2 fibres. The resting plasma lactate was mildly increased and there was elevated urinary excretion of lactic, pyruvic and acetoacetic acids, increased urinary excretion of carnitine esters, and increased plasma alanine. This pattern of metabolite excretion is consistent with an, as yet undefined, block in oxidative metabolism.