We report the discovery of extreme X-ray variability in a type 1 quasar: SDSS J$075101.42+291419.1$. It has a black hole mass of $1.6times 10^7~rm M_odot$ measured from reverberation mapping (RM), and the black hole is accreting with a super-Eddington accretion rate. Its XMM-Newton observation in 2015 May reveals a flux drop by a factor of $sim 22$ with respect to the Swift observation in 2013 May when it showed a typical level of X-ray emission relative to its UV/optical emission. The lack of correlated UV variability results in a steep X-ray-to-optical power-law slope ($alpha_{rm OX}$) of -1.97 in the low X-ray flux state, corresponding to an X-ray weakness factor of 36.2 at rest-frame 2 keV relative to its UV/optical luminosity. The mild UV/optical continuum and emission-line variability also suggest that the accretion rate did not change significantly. A single power-law model modified by Galactic absorption describes well the $0.3-10$ keV spectra of the X-ray observations in general. The spectral fitting reveals steep spectral shapes with $Gammaapprox3$. We search for active galactic nuclei (AGNs) with such extreme X-ray variability in the literature and find that most of them are narrow-line Seyfert 1 galaxies and quasars with high accretion rates. The fraction of extremely X-ray variable objects among super-Eddington accreting AGNs is estimated to be $approx 15-24%$. We discuss two possible scenarios, disk reflection and partial covering absorption, to explain the extreme X-ray variability of SDSS J$075101.42+291419.1$. We propose a possible origin for the partial covering absorber, which is the thick inner accretion disk and its associated outflow in AGNs with high accretion rates.