Myasthenia gravis (MG) as a presenting feature of extrathymic tumors are rarely reported. Here, we present a case of hepatocellular carcinoma (HCC) presented with MG. A 66-year-old Thai man presented to King Chulalongkorn Memorial hospital in September 2017 with weakness of his neck and legs. He also had trouble in swallowing and his voiced changed. The severity of those symptoms varied during a day while progressed over the past six months. Physical examination revealed weakness of neck muscle and the proximal muscle of extremities. Fatigability and enhanced ptosis were also observed. Electromyography showed a reproducible decremental response with repetitive nerve stimulation. High level of AChR-IgG (17.9 nmol/L) was compatible with a diagnosis of MG. The chest CT revealed no thymoma. However, the abdominal CT (figure 1) showed cirrhotic features with a 5.1x3.1 cm arterial enhancing and rapid venous washout mass in hepatic segment VIII invading segmental branches of right portal vein, representing the typical radiologic characteristics of HCC. The alpha-fetoprotein was 455 IU/mL. Serum immunologic test were negative for hepatitis B and C. The controlled attenuation parameter measured by transient elastography was 271 dB/m. The final diagnosis was fatty liver with cirrhosis grading Child-Pugh class A with HCC Barcelona Clinic Liver Cancer (BCLC) stage C and MG. The weakness improved after a treatment with intravenous immunoglobulin. Other medications were oral prednisolone 30 mg/d and pyridostigmine 240 mg/d which could be gradually tapered over time. Meanwhile, HCC was treated with two sessions of transarterial radioembolization (TARE) with Yttrium-90. The follow-up abdominal MRI (figure 2), 6 weeks after the 2nd TARE, demonstrated a decrease in size of both the HCC and the tumor thrombus in right portal vein. Several studies have been reported a high incidence of extrathymic malignancies with or without thymoma in MG patients, especially in the older age group. The pathophysiologic mechanism, even inconclusive, is suggested to be a paraneoplastic syndrome. However, there is no association between MG and any specific cancers. Approximately ten percent of these malignancies, with a rare report of HCC, present simultaneously with the MG. This case could be considered as the paraneoplastic MG, as an initial presentation, in a patient with advanced stage HCC. However, their associations require further investigations.2405_A Figure 1. The axial view of abdominal CT showed a 5.1x3.1 cm hypodensity mass in hepatic segment VIII (figure 1A; white arrow) with arterial enhancement in the arterial phase (figure1B) and rapid washout in the portovenous and delay phase (figure 1C and 1D, respectively) representing a typical radiologic features of HCC according to AASLD criteria.2405_B Figure 2. The axial view of the follow-up abdominal MRI (T1 dynamic phase) showed a decrease in size of HCC, 1.4x2.2 cm, in hepatic segment VIII (figure 2A; white arrow) without arterial enhancement in the arterial phase (figure 2B), representing responsiveness after TARE. Ring enhancement was observed in the portovenous and delay phase (figure 2C and 2D, respectively) representing fibrous tissue following TARE.