ABSTRACT Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder characterized by a deficiency of GPI-anchored proteins on blood cells. It is associated with hemolysis, thromboembolic events, and bone marrow failure. Management includes complement inhibitors, anticoagulation, and stem cell transplantation. Spontaneous remission of PNH is rarely reported. Case Presentation: A 28-year-old male initially diagnosed with aplastic anemia developed PVT and was subsequently diagnosed with PNH. Due to the unavailability of complement inhibitors, anticoagulation therapy and supportive care were employed. Splenectomy with splenorenal shunt was performed for chronic thrombosis and associated complications. The patient achieved spontaneous remission with normalized blood counts and diminished PNH clone. Conclusion: Managing PNH in resource-limited settings presents challenges due to the unavailability of complement inhibitors. Spontaneous remission of PNH is rarely reported and needs further research. Multidisciplinary approach, accessibility to diagnostic tests and advanced treatments will enhance PNH management in resource limited settings.

Keywords: Aplastic anemia, Paroxysmal nocturnal hemoglobinuria, Portal vein thrombosis, splenectomy, Resource limited countries.