Rhombencephalosynapsis is an exceptionally uncommon cerebellar anomaly characterized by the absence or underdevelopment of vermal axons, the presence of dentate nuclei, and the fusion of cerebral hemispheres. Depending on the presence or absence of additional supratentorial anomalies, the prognosis and clinical appearance can vary widely. Here, we present the case of a consanguineous-parent newborn boy, aged four days, who was diagnosed with the use of an MRI. The child had spastic diplegia, bone anomalies, and facial dysmorphism. Slight hydrocephalus, hypogenesis of the corpus callosum, and agenesis of the septum pellucidum were some of the additional supratentorial abnormalities. This study details the clinical and MRI findings, as well as a possible etiology, of this illness.