Fibromuscular dysplasia (FMD) is a rare condition that causes structural compromise of the blood vessel presenting either as an incidental radiological finding, dissection or stenosis usually of the renal or craniocervical arteries. Seldom, patients present with spontaneous dissection in visceral arteries and there are few reports of hepatic involvement. This report outlines the case of a 43-year-old female who presented with severe right upper quadrant pain with a subsequent diagnosis of FMD manifesting as spontaneous hepatic artery dissection. The patient was treated with conservative antiplatelet therapy and regular radiographic follow-up, decided by the treating team as no clear guidelines exist for management of this particular presentation of FMD. Surgical management is not currently recommended to this patient due to the risk of further dissection, but may be considered if there is severe haemodynamic compromise or refractory pain.