An experience with the diagnosis and treatment of patients with non-Oriental primary intrahepatic lithiasis (PIHL) is described. A group of 48 native Brazilian patients with symptomatic PIHL were studied, and the patients' characteristics, diagnoses, treatment protocols based on the presentation of the disease, prognostic factors, and late results were analyzed. Liver resection was performed in patients with an irreversible lesion, such as parenchymal atrophy or biliary stenosis; and biliary drainage procedures were employed in patients with bilateral disease. Late results were considered good when no postoperative symptoms were observed and poor if there was pain recurrence or cholangitis. Overall good results were observed in 73.4% of the patients. Good late results were observed in 94.1% and 62.1% of the patients with unilateral and bilateral stones, respectively. None of the analyzed parameters (gender, age, previous biliary surgery, bilirubin level, serum leukocyte counts, prothrombin activity, previous history of cholangitis, stone location) predicted poor late results, but the risk for patients with bilateral stones to develop late complications was 7.2 times higher than for those with unilateral disease. Non-Oriental PIHL is a rare disease, and the patients' characteristics are similar to those of patients with Oriental PIHL. We believe that personalized treatment based on the presentation of the disease led to the high incidence of good late results in this series.