Myasthenia gravis (MG) can rarely be manifested with ocular motility disturbances, simulating internuclear ophthalmoplegia. Pseudo-internuclear ophthalmoplegia (PINO) may occur during the course of MG, however, the initial presentation of MG with PINO in rather unlikely. We herein describe the case of a male patient who developed PINO, as an initial manifestation of MG.