Summary: Acute hepatic porphyrias (AHP) include acute intermittent porphyria, variegate porphyria, hereditary coproporphyria, and aminolevulinic acid dehydratase deficiency porphyria. Disorders of the heme synthesis pathway result in the overproduction of neurotoxic ALA and PBG. Clinically, recurrent episodes of severe abdominal pain without peritoneal signs predominate, often accompanied by autonomic disorders, electrolyte disturbances (especially hyponatremia), and neurological and psychiatric symptoms; in VP and HCP, skin lesions may coexist in areas exposed to UV light. In Poland, the patient population is estimated at 400–500 people, with a predominance of AIP and low clinical penetration, which results in difficulties in making a timely and correct diagnosis. A practical diagnostic algorithm for primary care professionals is based on quantitative determination of PBG and ALA in urine (preferably during an attack), with protection of the sample from light and appropriate transport logistics; results below 4× GGN justify diagnosis and urgent referral to a reference center. Management of an acute attack includes rapid rehydration and glucose supply, and in more severe cases, intravenous administration of heme, pain control, and treatment of complications. Prevention of attacks includes modification of triggering factors and pharmacological treatment in patients with frequent or severe attacks, including prophylactic administration of hemine, hormonal interventions in women, and givosiran, which has been available in Poland for several years under a drug program. The importance of early diagnosis, proper preservation of material, and rapid implementation of treatment to reduce neurological, renal, and hepatic complications was discussed.