Benign recurrent intrahepatic cholestasis (BRIC) is a rare inherited cholestatic disorder whose diagnosis is usually confirmed by genetic testing. However, access to molecular diagnostics remains limited in many low- and middle-income countries. Here, we report a 38-year-old Cambodian man presenting with his first episode of severe cholestatic jaundice and pruritus. Extensive investigations excluded infectious, autoimmune, metabolic, and obstructive causes. Liver biopsy reviewed by a French expert reference center demonstrated isolated cholestasis, supporting the diagnosis of BRIC despite the absence of genetic confirmation. The patient achieved complete clinical and biochemical remission during follow-up. This case illustrates that BRIC can be diagnosed with high confidence using established clinical criteria when genetic testing is unavailable and highlights the value of international collaboration in improving rare disease diagnosis in resource-limited settings.