Mixed connective tissue disease (MCTD) is a rare autoimmune condition that shows an overlap of at least two connective tissue diseases (CTD) including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), scleroderma, Sjögren's syndrome, polymyositis, or dermatomyositis. From a laboratory standpoint, MCTD is associated with high titers of anti-U1-RNP antibodies, which makes it difficult to determine whether it is a variant of each of the respective CTDs or a different entity altogether. Our objective is to report the case of a patient with MCTD presenting with status epilepticus who was ultimately diagnosed with lupus cerebritis.