Star fruit has been reported as containing neurotoxins that often cause severe neurological complications in patients with chronic renal disease. We report two patients with chronic renal failure at a pre-dialyzed stage who developed refractory status epilepticus after ingestion of star fruit. In addition, we review 51 cases in the literature. Among 53 patients, 16 patients presented with epileptic seizures (30%). The mortality rate was as high as 75% in patients with seizures. On the other hand, in patients without seizures, the mortality rate was only 0.03%. There is a poor correlation with the degree of underlying renal function and mortality due to intoxication. We propose that epileptic seizure is significantly associated with poor prognosis, and that status epilepticus is an unpredictable and potentially fatal complication in star fruit intoxication. We advise consultant neurologists that star fruit intoxication must be considered when patients with chronic renal disease present with seizures or other unexplained neurological or psychiatric symptoms. Since no effective treatment has been established, star fruit consumption should be avoided in patients with chronic renal disease, especially in the elderly.