Acute hepatic porphyrias (AHP) are rare metabolic diseases caused by enzyme defects along the heme biosynthesis pathway. In addition, in attacks the first and speed limiting enzyme ALAS1 is upregulated in liver cells. In patients with AHP, malnutrition with low caloric intake and moreover, some xenobiotics, such as herbs and spices added to meals, may induce hepatic ALAS1 transcription. Thus, diet may impact disease severity and quality of life in AHP. In this pilot study we focused on disease related dietary characteristics and body weight oscillations. Moreover, we report here on nutrition related triggers for porphyria attacks, body weight, stress-eating behaviour, and on how patients with AHP him-or herself perceive importance of diet in disease managing. In our Porphyria Centre a detailed questionnaire was applied to a cohort of 37 consecutive patients (30 females, 7 males, mean age of 45.4 years) with clinically and laboratory (urinary ALA and PBG, porphyrins) confirmed AHP. Notably, 13 of our patients reported specific foods such as garlic (24,3%), sauerkraut (10,8%) and alcohol (24, 3%), as triggers for attacks, and 31 modified their diet during an attack (avoid eating 30 or eating more foods rich in carbohydrates 10. Furthermore, 11, 18 and 8 of participants rated their diet as highly important, important, and not important, respectively. Additionally, 23 (62%) of patients paid attention to their diet also during latency phases. On average, patients gained 6.3 kilograms since the onset of the disease (mean of 50,6 months), with men gaining more weight (8.9 kilograms) compared to women (5.7 kilograms). Male patients exhibited a higher tendency to gain weight compared to female patients. Notably, 20 patients on Givlaari treatment for 24 months (range 3 to 66 months) reported a lower average weight gain (4,1 kg) compared to those not on the medication (8.9 kg), indicating towards to a potential metabolic stabilizing effect of Givlaari. Stress, defined as a physical and emotional response to situations perceived as threatening or challenging (APA, 2024), was reported to influence eating behaviours, with 20 of patients eating less and 11 eating more, often increasing their intake of sweets and fast food. These stress-induced dietary changes may lead to an unbalanced diet. When less eating, the risk of triggering porphyria attacks is increased. A substantial proportion of patients identified specific foods as potential triggers for porphyria attacks. Most patients modified their diet during attacks. Surprisingly, weight gain was common among patients with AHP, with men more than women. Notably, patients on Givlaari treatment experienced less weight gain, suggesting potential metabolic benefits of that medication. Stress-induced changes in eating behaviour further underscore the importance of dietary management in AHP. These findings suggest that comprehensive dietary counselling should be an integral part of managing AHP.