Background: Human immunodeficiency virus (HIV) type 2 infection is predominantly found in West African nations, and approximately 1 - 2 million people are thought to be infected. HIV-associated nephropathy (HIVAN) occurs in about 7% of patients with HIV-1 infection and is one of the most important causes of end-stage renal disease in this population. The only reported case of HIVAN related to HIV-2 infection was described by Izzedine et al. (2006). Aim: The aim of this paper is to report a case of HIVAN in an HIV-2-infected patient, a rarely described condition. Case presentation: We describe a case of a 40-year-old HIV-2-infected female from Angola hospitalized following a six-month history of fever, fatigue, anorexia and weight loss. Laboratory data revealed anaemia, leukopenia and renal dysfunction with nephrotic range proteinuria. Renal biopsy was performed, revealing findings consistent with HIVAN. Also, a presumed diagnosis of ganglionic tuberculosis was established. Conclusion: The slow progression of HIV-2 disease could explain the low frequency of this condition, however, more studies should be carried out for a better understanding of HIV-2 pathophysiology and its associated complications.