Scleroderma renal crisis with left ventricular diastolic dysfunction can lead to significant mortality. We presented the case of a 32-year-old female with anuria for 2 days. On further inquiry, she had joint pain, difficulty turning her head sidewise, and associated difficulty in finger movement. Also, hyperpigmentation with superimposed hypopigmentation was reported, which reduced during her pregnancy and worsened post-partum. Her family history suggested similar complaints in her mother. In addition, she had a blurring of vision. She had hypertension, microangiopathic hemolytic anemia, deranged renal function, and retinopathy on ophthalmologic examination. Radiological investigations revealed pulmonary edema, pleural effusion, and left ventricular diastolic dysfunction. Hence, a diagnosis of scleroderma renal crisis complicated by left ventricular diastolic dysfunction was made. She was managed conservatively using anti-hypertensive medications and hemodialysis, which resulted in gradual improvement. Our case highlighted the management approach to this rare presentation with anti-hypertensives and hemodialysis in a resource-limited setting.